The Patient with a Blood Disorder










The Patient with a
Blood Disorder
Lisa Welch, RDH, MSDH and Barbara Dawidjan, RDH, MED
NORMAL BLOOD
I. Composition
II. Origin of Blood Cells
PLASMA
RED BLOOD CELLS (ERYTHROCYTES)
I. Description
II. Functions
III. Hemoglobin
WHITE BLOOD CELLS (LEUKOCYTES)
I. Types of Leukocytes
II. Functions
III. Agranulocytes
IV. Granulocytes
PLATELETS (THROMBOCYTES)
ANEMIA
I. Classification by Cause
II. Clinical Characteristics of Anemia
IRON-DEFICIENCY ANEMIA
I. Characteristics
II. Causes
III. Signs and Symptoms
IV. Therapy
MEGALOBLASTIC ANEMIA
I. Pernicious Anemia
II. Folate-Deficiency Anemia
SICKLE CELL DISEASE
I. Disease Process
II. Clinical Course
III. Treatment and Disease Management
IV. Oral Implications
V. Appointment Management
POLYCYTHEMIAS
I. Polycythemia Vera (Primary Polycythemia)
II. Secondary Polycythemia
DISORDERS OF WHITE BLOOD CELLS
I. Neutropenia
II. Lymphocytopenia
III. Leukocytosis
PLATELET DISORDERS
I. Thrombocytopenia
II. Platelet Dysfunction
BLEEDING (OR COAGULATION) DISORDERS
I. Oral Findings Suggestive of Blood Disorders
II. Types of Disorders of Coagulation
DENTAL HYGIENE CARE PLAN
I. Preparation for Clinical Appointment
II. Patient History
III. Consultation with Physician/Hematologist
IV. Treatment
DOCUMENTATION
EVERYDAY ETHICS
FACTORS TO TEACH THE PATIENT
REFERENCES
CHAPTER OUTLINE
68
LEARNING OBJECTIVES
After studying this chapter, the student will be able to:
1. Describe the major types of blood disorders.
2. Explain the general and oral signs and symptoms of the
major types of blood disorders.
3. Identify clinical implications of selected blood values
including the INR (international normalized ratio), platelet
count, and neutrophil count.
4. Provide examples of dental hygiene treatment modifications
necessary for the patient with a blood disorder.
Wilkins9781451193114-ch068.indd 1147 07/10/15 11:40 AM

1148 SECTION IX | Patients with Special Needs
Oral soft tissue changes, lowered resistance to infection,
and bleeding tendencies are major factors to be considered
for a patient with a blood disorder. Oral manifestations of
blood disorders are generally exaggerated in the presence
of dental biofilm and local predisposing factors.
Box 68-1 lists and defines terminology used to describe
hematologic conditions.
NORMAL BLOOD
1
I. Composition
Blood is composed of 55% plasma fluid and 45% formed
elements.
The 45% formed elements consist of:
• 44% erythrocytes (red blood cells or corpuscles)
• 1% leukocytes (white blood cells).
Figure 68-1 shows the cell forms and nuclei.
Hematocrit, a test commonly used in health examina-
tions, shows:
• Percentage packed volume of blood cells
• Normal values are listed in Table 68-1.
Reference values for blood cells are also listed in
Table 68-1, with examples of conditions in which in-
creases and decreases in the normal values occur.
II. Origin of Blood Cells
Adult blood cells originate in bone marrow.
Hemocytoblast: the stem cell of origin.
Erythrocytes and granulocytes leave the bone marrow
as mature cells and enter the circulating blood.
Agranulocytes (lymphocytes and monocytes) leave the
bone marrow as immature cells and go to lymphoid tis-
sues for maturing.
Immature cell forms predominate in certain blood dis-
eases and cancers.
PLASMA
The constituents of the fluid portion of the blood are
similar to the fluid constituents of the connective tissue.
If plasma is allowed to clot, the remaining fluid is called
serum.
The plasma is composed of 90% water and 10% of the
following:
Plasma proteins
• Albumin (functions to maintain tissue fluid balance
within the vascular system).
• Gamma globulins (circulating antibodies essential
to the immune system).
Chelation therapy: process to remove excess iron acquired
from chronic blood transfusions.
Coagulation factor: factor essential to normal blood clotting
contained within the blood plasma; designated by Roman
numerals I–V and VII–XIII; their absence, diminution, or ex-
cess may lead to abnormality of clotting.
Differential cell count: record of the number of white blood
cells, including determination of the percentage of each
type of cell present; the differential” is used in the diagno-
sis of various blood disorders, infections, and other abnor-
mal conditions of the body.
Erythropoiesis: formation of red blood cells.
Glossitis: inflammation of the tongue.
Glossodynia: pain in the tongue.
Hemarthrosis: blood in a joint cavity.
Hematocrit: volume percentage of erythrocytes (red blood
cells) in whole blood.
Hematopoiesis: the formation and development of blood
cells, usually in bone marrow.
Hemoglobin: protein in the erythrocyte that transports mo-
lecular oxygen to body cells.
Hemolysis: rupture of erythrocytes with the release of hemo-
globin into the plasma.
Hemolytic: destruction of blood cells, resulting in liberation
of hemoglobin.
Hypoxia: diminished availability of oxygen to body tissues.
IF (Intrinsic Factor): produced by the parietal cells in the
stomach; aids in vitamin B 12 absorption.
INR (International Normalized Ratio): ratio between actual
blood coagulation time and the normal coagulation time.
Leukocytosis: increase in the total number of leukocytes.
Leukopenia: reduction in total number of leukocytes in the
blood; count under 500 per mL.
Neutropenia: diminished number of neutrophils (polymor-
phonuclear leukocytes or PMNs).
Oxyhemoglobin: oxygenated arterial blood; bright red and
about 97% saturated with oxygen; venous blood is a
darker color and contains only 20%–70% oxygen.
Petechia: minute, pinpoint, round, nonraised, purplish-
red spot in the skin or mucous membrane, caused by
hemorrhage.
Phagocytosis: engulfing of microorganisms and foreign par-
ticles by phagocytes, such as macrophages.
Purpura: hemorrhage into the tissues, under the skin, and
through the mucous membranes; produces petechiae and
ecchymoses.
Vaso-occlusion: blood vessel blockage resulting in organ
damage.
Vertigo: dizziness.
BOX 68-1 KEY WORDS: Bleeding Disorders
Wilkins9781451193114-ch068.indd 1148 07/10/15 11:40 AM

CHAPTER 68 | The Patient with a Blood Disorder 1149
FIGURE 68-1 Red and White Blood Cells. Diagram shows normal cell forms drawn to scale for comparison of cell size. Note the shape of nuclei in each of the white blood cells. The
erythrocyte or red blood cell does not have a nucleus; its biconcave disc shape is shown in the lateral view second from the left.
TABLE 68-1 Laboratory Values and Clinical Implications
TEST NORMAL RANGE
a
CLINICAL IMPLICATIONS CAUSES OF DEVIATIONS
PT (prothrombin
time)
11–15 sec Routine care can be performed when PT
is <20 sec
Prolonged in:
Prothrombin deficiency
Anticoagulant therapy
Vitamin K deficiency
Liver diseases
Aspirin use
INR
<2.5
Routine care can be performed when INR
2–3, MD consult when INR > 3.0
Prolonged in:
Polycythemia vera (PV)
Prothrombin deficiency
Anticoagulant therapy
Vitamin K deficiency
Liver diseases
Aspirin use
Activated partial
thromboplastin
time (aPTT)
25–35 sec
Routine care when aPTT is <1.5 ×
normal, MD consult when > 57 sec
Prolonged in:
Hemophilia and von Willebrand’s disease
Anticoagulant therapy
Platelet count 140,000–400,000/mm
3
Routine care can be provided when values
are >50,000/mm
3
MD consult needed when values <10,000/
mm
3
—potentially life threatening
Thrombocytopenia:
<20,000 mm
3
Hemoglobin (g/dL) Males: 13.6–17.2 g/100
mL
Females: 12–15 g/100
mL
Delivers O
2
through circulation to body
tissues and returns CO
2
from tissues to
lungs
Increased in:
Polycythemia
Dehydration
Decreased in:
Anemias
Hemorrhage
Leukemias
Hematocrit
(volume of
packed red cells)
(percentage)
Males: 39%–49%
Females: 33%–43%
Indicates relative proportions of plasma
and red blood cells
Increased in:
Polycythemia
Dehydration
Decreased in:
Anemias
Hemorrhage leukemias
Absolute neutrophil
count (ANC)
Normal ANC:
2,500–6,000
Measure of the number of infection
fighting white blood cells.
Routine care 50,000/mm
3
<500/mm
3
potentially life threatening
Decreased in:
Anemias
Chemotherapy
a
Ranges vary among health facilities and laboratories. The reference ranges of the facility providing the results are used in interpreting the test result.
Wilkins9781451193114-ch068.indd 1149 07/10/15 11:40 AM

1150 SECTION IX | Patients with Special Needs
II. Functions
Phagocytic, immunologic, and other functions related
to the inflammatory process in the connective tissue.
They pass through the walls at the terminal ends of cap-
illaries and into the connective tissue.
A large number of cells migrate into the area of injury.
Neutrophils arrive first and are active in the phagocyto-
sis of foreign material and microorganisms.
Blood functions as a transport medium for the white
cells as they pass to areas in the connective tissue where
they are needed.
Numbers and proportions in the blood maintain a con-
stant level in health, as listed in Table 68-2.
Differential cell count of the white blood cells is used
in the detection and monitoring of disease states. In-
creases and decreases of each cell type can be associated
with certain conditions.
III. Agranulocytes
Lymphocytes
• Small round cells with a round nucleus that nearly
fills the cell with a narrow rim of cytoplasm (Figure
68-1).
• Can move back and forth between the vessels and
the extravascular tissues.
• Capable of reverting to blast-like cells of origin and
then multiplying as the immunologic need arises.
Monocytes
• Large cells with a bean-shaped or indented nucleus.
• Actively phagocytic.
• In connective tissue, monocytes differentiate into
macrophages, which are important in immunologic
processes.
IV. Granulocytes
Neutrophils
• Also called polymorphonuclear leukocytes (PMNs).
• Most numerous of all the white blood cells.
• Nucleus has three to five lobes connected by thin
chromatin threads.
• Cells are round in circulation.
• They are amoeboid (moving and changing shape)
in the tissues and function in phagocytosis. Neutro-
phils are part of the first line of defense of the body.
Eosinophils
• Two-lobed nucleus and larger, coarser granules than
those of a neutrophil.
• Microscopically, the cells stain a distinct bright
pink; are readily recognized.
• Few in number; increase markedly during allergic
conditions.
• Beta globulins (aid in transport of hormones, metal-
lic ions, and lipids).
• Fibrinogen and prothrombin (essential for blood
clotting).
Inorganic salts include: sodium, potassium, calcium, bi-
carbonate, and chloride.
Gases include: dissolved oxygen, carbon dioxide, and
nitrogen.
Substances being transported include: hormones, nutri-
ents, waste products, and enzymes.
RED BLOOD CELLS (ERYTHROCYTES)
I. Description
Although commonly called red blood cells, they are
more properly termed corpuscles because they have no
nuclei (Figure 68-1).
Biconcave discs that contain hemoglobin.
Sensitive and flexible; change shape readily as they pass
through small capillaries.
II. Functions
Transport hemoglobin.
Carry oxygen to the body cells in the form of
oxyhemoglobin.
Carbon dioxide is transported from the cells.
III. Hemoglobin
Measured in grams (g) per 100 milliliters (mL).
Normal values range from 12 to 17.2 g per mL depend-
ing on gender (Table 68-1).
Values reflect:
• Anemic state when hemoglobin is below normal.
• Pathologic conditions when hemoglobin increases
over normal.
WHITE BLOOD CELLS (LEUKOCYTES)
I. Types of Leukocytes
White blood cells are divided into two general groups,
the granulocytes and the agranulocytes.
Granulocytes have granules in their cytoplasm, whereas
the agranulocytes do not.
They are further subdivided into the following
categories:
• Granulocytes: neutrophils, eosinophils, and
basophils.
• Agranulocytes: lymphocytes, monocytes.
Wilkins9781451193114-ch068.indd 1150 07/10/15 11:40 AM

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The Patient with a Blood DisorderLisa Welch, RDH, MSDH and Barbara Dawidjan, RDH, MEDNORMAL BLOOD I. Composition II. Origin of Blood CellsPLASMARED BLOOD CELLS (ERYTHROCYTES) I. Description II. Functions III. HemoglobinWHITE BLOOD CELLS (LEUKOCYTES) I. Types of Leukocytes II. Functions III. Agranulocytes IV. GranulocytesPLATELETS (THROMBOCYTES)ANEMIA I. Classification by Cause II. Clinical Characteristics of AnemiaIRON-DEFICIENCY ANEMIA I. Characteristics II. Causes III. Signs and Symptoms IV. TherapyMEGALOBLASTIC ANEMIA I. Pernicious Anemia II. Folate-Deficiency AnemiaSICKLE CELL DISEASE I. Disease Process II. Clinical Course III. Treatment and Disease Management IV. Oral Implications V. Appointment ManagementPOLYCYTHEMIAS I. Polycythemia Vera (Primary Polycythemia) II. Secondary PolycythemiaDISORDERS OF WHITE BLOOD CELLS I. Neutropenia II. Lymphocytopenia III. LeukocytosisPLATELET DISORDERS I. Thrombocytopenia II. Platelet DysfunctionBLEEDING (OR COAGULATION) DISORDERS I. Oral Findings Suggestive of Blood Disorders II. Types of Disorders of CoagulationDENTAL HYGIENE CARE PLAN I. Preparation for Clinical Appointment II. Patient History III. Consultation with Physician/Hematologist IV. TreatmentDOCUMENTATIONEVERYDAY ETHICSFACTORS TO TEACH THE PATIENTREFERENCESCHAPTER OUTLINE68LEARNING OBJECTIVESAfter studying this chapter, the student will be able to:1. Describe the major types of blood disorders.2. Explain the general and oral signs and symptoms of the major types of blood disorders.3. Identify clinical implications of selected blood values including the INR (international normalized ratio), platelet count, and neutrophil count.4. Provide examples of dental hygiene treatment modifications necessary for the patient with a blood disorder.Wilkins9781451193114-ch068.indd 1147 07/10/15 11:40 AM 1148 SECTION IX | Patients with Special NeedsOral soft tissue changes, lowered resistance to infection, and bleeding tendencies are major factors to be considered for a patient with a blood disorder. Oral manifestations of blood disorders are generally exaggerated in the presence of dental biofilm and local predisposing factors.Box 68-1 lists and defines terminology used to describe hematologic conditions.NORMAL BLOOD1I. Composition ▶ Blood is composed of 55% plasma fluid and 45% formed elements. ▶ The 45% formed elements consist of:• 44% erythrocytes (red blood cells or corpuscles)• 1% leukocytes (white blood cells). ▶ Figure 68-1 shows the cell forms and nuclei. ▶ Hematocrit, a test commonly used in health examina-tions, shows:• Percentage packed volume of blood cells• Normal values are listed in Table 68-1. ▶ Reference values for blood cells are also listed in Table 68-1, with examples of conditions in which in-creases and decreases in the normal values occur.II. Origin of Blood Cells ▶ Adult blood cells originate in bone marrow. ▶ Hemocytoblast: the stem cell of origin. ▶ Erythrocytes and granulocytes leave the bone marrow as mature cells and enter the circulating blood. ▶ Agranulocytes (lymphocytes and monocytes) leave the bone marrow as immature cells and go to lymphoid tis-sues for maturing. ▶ Immature cell forms predominate in certain blood dis-eases and cancers.PLASMA ▶ The constituents of the fluid portion of the blood are similar to the fluid constituents of the connective tissue. ▶ If plasma is allowed to clot, the remaining fluid is called serum. ▶ The plasma is composed of 90% water and 10% of the following: ▶ Plasma proteins• Albumin (functions to maintain tissue fluid balance within the vascular system).• Gamma globulins (circulating antibodies essential to the immune system).Chelation therapy: process to remove excess iron acquired from chronic blood transfusions.Coagulation factor: factor essential to normal blood clotting contained within the blood plasma; designated by Roman numerals I–V and VII–XIII; their absence, diminution, or ex-cess may lead to abnormality of clotting.Differential cell count: record of the number of white blood cells, including determination of the percentage of each type of cell present; the “differential” is used in the diagno-sis of various blood disorders, infections, and other abnor-mal conditions of the body.Erythropoiesis: formation of red blood cells.Glossitis: inflammation of the tongue.Glossodynia: pain in the tongue.Hemarthrosis: blood in a joint cavity.Hematocrit: volume percentage of erythrocytes (red blood cells) in whole blood.Hematopoiesis: the formation and development of blood cells, usually in bone marrow.Hemoglobin: protein in the erythrocyte that transports mo-lecular oxygen to body cells.Hemolysis: rupture of erythrocytes with the release of hemo-globin into the plasma.Hemolytic: destruction of blood cells, resulting in liberation of hemoglobin.Hypoxia: diminished availability of oxygen to body tissues.IF (Intrinsic Factor): produced by the parietal cells in the stomach; aids in vitamin B 12 absorption.INR (International Normalized Ratio): ratio between actual blood coagulation time and the normal coagulation time.Leukocytosis: increase in the total number of leukocytes.Leukopenia: reduction in total number of leukocytes in the blood; count under 500 per mL.Neutropenia: diminished number of neutrophils (polymor-phonuclear leukocytes or PMNs).Oxyhemoglobin: oxygenated arterial blood; bright red and about 97% saturated with oxygen; venous blood is a darker color and contains only 20%–70% oxygen.Petechia: minute, pinpoint, round, nonraised, purplish-red spot in the skin or mucous membrane, caused by hemorrhage.Phagocytosis: engulfing of microorganisms and foreign par-ticles by phagocytes, such as macrophages.Purpura: hemorrhage into the tissues, under the skin, and through the mucous membranes; produces petechiae and ecchymoses.Vaso-occlusion: blood vessel blockage resulting in organ damage.Vertigo: dizziness.BOX 68-1 KEY WORDS: Bleeding DisordersWilkins9781451193114-ch068.indd 1148 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1149FIGURE 68-1 Red and White Blood Cells. Diagram shows normal cell forms drawn to scale for comparison of cell size. Note the shape of nuclei in each of the white blood cells. The erythrocyte or red blood cell does not have a nucleus; its biconcave disc shape is shown in the lateral view second from the left.TABLE 68-1 Laboratory Values and Clinical ImplicationsTEST NORMAL RANGEaCLINICAL IMPLICATIONS CAUSES OF DEVIATIONSPT (prothrombin time)11–15 sec Routine care can be performed when PT is <20 secProlonged in: Prothrombin deficiency Anticoagulant therapy Vitamin K deficiency Liver diseases Aspirin useINR<2.5Routine care can be performed when INR 2–3, MD consult when INR > 3.0Prolonged in: Polycythemia vera (PV) Prothrombin deficiency Anticoagulant therapy Vitamin K deficiency Liver diseases Aspirin useActivated partial thromboplastin time (aPTT)25–35 secRoutine care when aPTT is <1.5 × normal, MD consult when > 57 secProlonged in: Hemophilia and von Willebrand’s disease Anticoagulant therapyPlatelet count 140,000–400,000/mm3Routine care can be provided when values are >50,000/mm3MD consult needed when values <10,000/ mm3—potentially life threateningThrombocytopenia: <20,000 mm3Hemoglobin (g/dL) Males: 13.6–17.2 g/100 mLFemales: 12–15 g/100 mLDelivers O2 through circulation to body tissues and returns CO2 from tissues to lungsIncreased in: Polycythemia DehydrationDecreased in: Anemias Hemorrhage LeukemiasHematocrit (volume of packed red cells) (percentage)Males: 39%–49%Females: 33%–43%Indicates relative proportions of plasma and red blood cellsIncreased in: Polycythemia DehydrationDecreased in: Anemias Hemorrhage leukemiasAbsolute neutrophil count (ANC)Normal ANC: 2,500–6,000Measure of the number of infection fighting white blood cells.Routine care 50,000/mm3 <500/mm3— potentially life threateningDecreased in: Anemias ChemotherapyaRanges vary among health facilities and laboratories. The reference ranges of the facility providing the results are used in interpreting the test result.Wilkins9781451193114-ch068.indd 1149 07/10/15 11:40 AM 1150 SECTION IX | Patients with Special NeedsII. Functions ▶ Phagocytic, immunologic, and other functions related to the inflammatory process in the connective tissue. ▶ They pass through the walls at the terminal ends of cap-illaries and into the connective tissue. ▶ A large number of cells migrate into the area of injury. ▶ Neutrophils arrive first and are active in the phagocyto-sis of foreign material and microorganisms. ▶ Blood functions as a transport medium for the white cells as they pass to areas in the connective tissue where they are needed. ▶ Numbers and proportions in the blood maintain a con-stant level in health, as listed in Table 68-2. ▶ Differential cell count of the white blood cells is used in the detection and monitoring of disease states. In-creases and decreases of each cell type can be associated with certain conditions.III. Agranulocytes ▶ Lymphocytes• Small round cells with a round nucleus that nearly fills the cell with a narrow rim of cytoplasm (Figure 68-1).• Can move back and forth between the vessels and the extravascular tissues.• Capable of reverting to blast-like cells of origin and then multiplying as the immunologic need arises. ▶ Monocytes• Large cells with a bean-shaped or indented nucleus.• Actively phagocytic.• In connective tissue, monocytes differentiate into macrophages, which are important in immunologic processes.IV. Granulocytes ▶ Neutrophils• Also called polymorphonuclear leukocytes (PMNs).• Most numerous of all the white blood cells.• Nucleus has three to five lobes connected by thin chromatin threads.• Cells are round in circulation.• They are amoeboid (moving and changing shape) in the tissues and function in phagocytosis. Neutro-phils are part of the first line of defense of the body. ▶ Eosinophils• Two-lobed nucleus and larger, coarser granules than those of a neutrophil.• Microscopically, the cells stain a distinct bright pink; are readily recognized.• Few in number; increase markedly during allergic conditions.• Beta globulins (aid in transport of hormones, metal-lic ions, and lipids).• Fibrinogen and prothrombin (essential for blood clotting). ▶ Inorganic salts include: sodium, potassium, calcium, bi-carbonate, and chloride. ▶ Gases include: dissolved oxygen, carbon dioxide, and nitrogen. ▶ Substances being transported include: hormones, nutri-ents, waste products, and enzymes.RED BLOOD CELLS (ERYTHROCYTES)I. Description ▶ Although commonly called red blood cells, they are more properly termed corpuscles because they have no nuclei (Figure 68-1). ▶ Biconcave discs that contain hemoglobin. ▶ Sensitive and flexible; change shape readily as they pass through small capillaries.II. Functions ▶ Transport hemoglobin. ▶ Carry oxygen to the body cells in the form of oxyhemoglobin. ▶ Carbon dioxide is transported from the cells.III. Hemoglobin ▶ Measured in grams (g) per 100 milliliters (mL). ▶ Normal values range from 12 to 17.2 g per mL depend-ing on gender (Table 68-1). ▶ Values reflect:• Anemic state when hemoglobin is below normal.• Pathologic conditions when hemoglobin increases over normal.WHITE BLOOD CELLS (LEUKOCYTES)I. Types of Leukocytes ▶ White blood cells are divided into two general groups, the granulocytes and the agranulocytes. ▶ Granulocytes have granules in their cytoplasm, whereas the agranulocytes do not. ▶ They are further subdivided into the following categories:• Granulocytes: neutrophils, eosinophils, and basophils.• Agranulocytes: lymphocytes, monocytes.Wilkins9781451193114-ch068.indd 1150 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1151TABLE 68-2 Blood Cells Reference ValuesAn examination of a blood smear (or film) may be requested by a physician in response to abnormality in blood counts.CELL TYPE NORMAL VALUE CAUSES OF INCREASE CAUSES OF DECREASERed blood cells (erythrocytes) Males: 4.3–5.9 million per mm3Females: 3.5–5.0 million per mm3Polycythemia dehydration AnemiasLeukemiasHemorrhagePlatelets (thrombocytes) (cell fragments essential for the process of blood clotting)150,000–400,000 per mm3Wintrobe method: 140,000–440,000 per mm3Polycythemia vera (PV)Chronic myelocytic leukemiaSickle cell anemiaRheumatic feverHemolytic anemiasBone fracturesAcute severe infectionsCirrhosis of the liverThrombocytopenic purpuraAcute leukemiasAplastic anemiasPernicious anemiaWhite blood cells (leukocytes)5,000–10,000 per mm3InflammationOverexertionPolycythemia vera (PV)LeukemiaAplastic anemiaGranulocytopeniaDrug poisoningThrombocytopeniaRadiationSevere infections HIV/AIDSDifferential white cell count granulocytes• Neutrophils• Eosinophils• Basophils60%–70%1%–3%1%Acute infectionsMyelogenous leukemiaPoisoning ErythroblastosisAllergic diseases DermatitisHodgkin’s diseaseScarlet feverCertain chronic infectionsAplastic anemiaGranulocytopeniaAplastic anemiaTyphoid feverAplastic anemiaAgranulocytes• Lymphocytes• Monocytes20%–35%2%–6%Lymphocytic leukemiaChronic infectionsViral diseasesMonocytic leukemiasTuberculosisInfective endocarditisHodgkin’s diseaseAplastic anemiaMyelogenous leukemiaRadiationAplastic anemia ▶ Basophils• Nucleus has a “U” or “S” form.• Function is to increase vascular permeability during inflammation so that phagocytic cells can pass into the area.PLATELETS (THROMBOCYTES) ▶ Small round or oval formed element without a nucleus ▶ Approximately one-fourth the size of a red blood cell. ▶ Active in the blood clotting mechanism. ▶ Essential in the maintenance of the integrity of blood capillaries by repairing them at the time of injury. ▶ Participate in clot dissolution after healing.ANEMIA ▶ Anemia is a reduction in red blood cells to a level be-low a normal level for the individual.2 ▶ Oxygen-carrying capacity to the cells is diminished.I. Classification by CauseA. Caused by Blood Loss ▶ Acute: Blood loss from trauma or disease ▶ Chronic: An internal lesion with constant slow bleed-ing, usually of gastrointestinal or gynecologic origin, can lead to a chronic loss of blood. Iron-deficiency ane-mia can result.Wilkins9781451193114-ch068.indd 1151 07/10/15 11:40 AM 1152 SECTION IX | Patients with Special Needs ▶ The most severe form is beta thalassemia major (Cooley’s anemia). ▶ Blood counts are evaluated and monitored for delayed wound healing. ▶ Treatment4• May require periodic and lifelong blood transfusions and chelation therapy.• Folic acid supplements.• If performed during childhood, bone marrow trans-plant is a potential cure.• Hematopoietic (blood forming) stem-cell transplan-tation is the only available treatment for thalassemia.II. Clinical Characteristics of Anemia2When a patient’s medical history shows the presence of anemia, certain general characteristics may be anticipated for which clinical adaptations may be needed. The general signs and symptoms are: ▶ Pale skin, nails, buccal mucosa ▶ Weakness, malaise, easy fatigability ▶ Dyspnea on slight exertion, faintness ▶ Brittle nails with loss of convexity referred to as spoon-ing of the nails.IRON-DEFICIENCY ANEMIA5,6I. CharacteristicsIron-deficiency anemia is a hypochromic microcytic ane-mia, which means that: ▶ Hemoglobin content is deficient (hypochromic) ▶ Red blood cells are smaller than normal (microcytic) ▶ Occurs more in younger than older people and more in females than in males ▶ Diagnosis is by laboratory test that shows low hemoglo-bin and a reduced hematocrit value.II. Causes ▶ Malnutrition, poor dietary intake, or malabsorption (e.g., celiac disease and Crohn’s disease). ▶ Chronic infection. ▶ Bariatric surgery (gastric bypass). ▶ Increased body demand for iron over and above the daily intake. For example, during pregnancy. ▶ Acute or chronic blood loss due to:• Excessive menstrual flow.• Frequent blood donations. ▶ Internal bleeding due to:• Gastrointestinal diseases, such as ulcer and colon or stomach cancer.B. Caused by Increased HemolysisHemolysis means the destruction of red blood cells; is also called “hemolytic anemia” because of cell destruction. ▶ Hereditary hemolytic disorders• Sickle cell disease (SCD), which belongs to the group of hereditary disorders called the hemoglobinopathies ▶ Acquired hemolytic disorders• Examples: drugs, infections, and certain physi-cal and chemical agents may cause red cell destruction.• In the category of antibody-mediated anemia, eryth-roblastosis fetalis (also called hemolytic disease of the newborn) occurs when an expectant mother is Rh positive and the fetus is Rh negative.C. Caused by Diminished Production of Red Blood Cells ▶ Nutritional deficiency• Inadequate dietary choices or inadequate intake.• Defective absorption from the gastrointestinal tract.• Examples: pernicious anemia, which results from a B12 vitamin absorption deficiency, iron-deficiency ane-mia, which may occur during pregnancy or during a growth spurt, and celiac disease (sprue), which results from sensitivity to dietary gluten.• Increased demand for nutrients such as in growth or pregnancy. ▶ Bone marrow failure• Aplastic anemia (which can be inherited) can occur without apparent cause or when the bone marrow is injured by medications, radiation, chemotherapy, or infection.• In aplastic anemia, a combination of anemia, neu-tropenia, and thrombocytopenia occurs, which leads to a quantitative decrease in all cells formed in the bone marrow.• Consult with physician to determine if antibiotic premedication would be indicated.D. Anemia of Chronic Diseases3 ▶ Second most prevalent anemia after iron-deficiency anemia. ▶ Many chronic systemic diseases are associated with anemia.E. Caused by Genetic Blood Disorders ▶ Thalassemia is a diverse group of genetic blood disor-ders characterized by defects in synthesis of normal hemoglobin.4 ▶ It typically affects people of Mediterranean, African, Middle Eastern, and Southeast Asian descent. ▶ The condition can range in severity from mild to life threatening.Wilkins9781451193114-ch068.indd 1152 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1153• Deficiency of vitamin B12 can also be caused by:1. Malabsorption of vitamin B12 due to gastrointestinal disorders such as celiac disease and Crohn’s disease.2. Inadequate intake may occur in strict vegans (those who do not eat foods from animal sources, for example, dairy or meat).3. Chronic gastritis or surgical removal of part or all of the stomach (e.g., gastric bypass) can reduce or stop the production of IF.4. Long-term use of histamine (H2) receptor antago-nists, proton pump inhibitors, or metformin. ▶ Age characteristics• Pernicious anemia is primarily a disease of adults and occurs with increasing frequency in those over age60. ▶ Signs and symptoms: general• Fatigue and weakness.• Loss of appetite and weight loss.• Poor memory. ▶ Signs and symptoms: neurologic involvement• Sclerosis of the spinal cord.• Polyneuritis (inflammation or degeneration of nerves causing pain).• Numbness of hands and feet.• Ataxia (loss of coordination). ▶ Signs and symptoms: oral findings• Glossitis (Hunter’s glossitis) slick or bald tongue, loss of filiform papillae, and burning sensation with certain foods.• Sensitivity to hot or spicy foods.• Gingiva and mucosa: pale, atrophic similar to vita-min B deficiency. ▶ Treatment• For individuals with IF deficiency and/or malabsorp-tion, vitamin B12 is administered by injection daily until the condition is controlled.• Once controlled, administration will continue monthly for life or until the underlying condition causing the deficiency is managed.• For patients with inadequate IF production, oral sup-plementation of vitamin B12 is the treatment of choice.• Vitamin B12 is only found in foods from animal sources and fortified foods. Good dietary sources of vitamin B12 are meat, clams, liver, fortified breakfast cereals, fish, poultry, milk, cheese, and eggs.II. Folate-Deficiency Anemia10Folate-deficiency anemia has the same characteristics as pernicious anemia, except no clinical neurologic changes are evident. ▶ Etiologic factors• Decreased intake.• Drugs, notably aspirin.• Hemorrhoids.• Chronic alcoholism.III. Signs and Symptoms ▶ General• General weakness, pallor• Fatigue on slight exertion• Decreased immune function and increased risk for infection ▶ Oral findings• Pallor of the mucosa and gingiva• Tongue changes: atrophic glossitis with loss of fili-form papillae. In moderate and severe anemia, when the hemoglobin is at 10 g/dL or below, the tongue is smooth and shiny. The patient may have burning, painful sensations (glossodynia).• Secondary irritations to the thinned, atrophic mu-cosa may result from smoking, mechanical trauma, or hot, spicy foods.• Angular cheilitis.• Increased risk of candidiasis.IV. Therapy ▶ Treatment of underlying cause to prevent further blood loss. ▶ Treated with oral ferrous iron tablets with vitamin C to aid absorption; to be taken on empty stomach for best absorption rates. ▶ Folic acid supplements may be indicated if there is an underlying folate deficiency. ▶ Nutritional counseling: recommend foods high in iron. Sources given in Chapter 35. ▶ Liquid preparations, which are sometimes used for chil-dren, may stain the teeth. Administering the medicine by way of a straw is advised.MEGALOBLASTIC ANEMIACharacterized by abnormally large (megalo-) red blood cells, many of which are oval shaped resulting from a defi-ciency of either vitamin B12 or folate, or both.I. Pernicious Anemia7–9 ▶ Etiologic factors• Pernicious anemia is considered to be an autoim-mune disease affecting the gastric mucosa so that it atrophies and fails to produce intrinsic factor (IF).• IF is excreted by the stomach parietal cells and is necessary for absorption of vitamin B12.Wilkins9781451193114-ch068.indd 1153 07/10/15 11:40 AM 1154 SECTION IX | Patients with Special NeedsI. Disease ProcessOccurs primarily in the African-American population and in white populations of Mediterranean origin. ▶ Diagnosis• Prenatal diagnosis can be made with 100% accuracy and genetic counseling provided for the parents.• A simple blood test will detect SCD or sickle cell trait (parental carrier). Newborn screening is man-datory in most states in the United States.II. Clinical Course13 ▶ Anemia• Life span of red blood cells is significantly reduced from a normal lifespan of approximately 90–120 days, to about 10–15 days.• Anemia starts appearing within the first 6 months.• Growth and development may be impaired during the early years.• Increased susceptibility to infection and especially to pneumococcal infections. ▶ Vaso-occlusion• Mortality is primarily from repeated episodes of vaso-occlusion (or blood vessel blockage) resulting in infarctions causing stroke and organ damage.• There are progressive changes to blood vessels as a result of damage by the sickle-shaped red blood cells that result in the blood vessel blockage.• Organ damage may be progressive and result in a shortened life expectancy, with most patients only living into their 30s. ▶ Pain crises (sickle cell crisis)• Repeated episodes of acute pain that are highly un-predictable and result in hospitalization.• Often preceded by a viral or bacterial infection. ▶ Chronic organ damageOver time most sickle cell patients have damage to organs.• Neurologic damage results in impaired cognition and hemorrhagic strokes.• Cardiopulmonary damage impairs lung function and results in dilation of the left ventricle of the heart and heart murmurs.• Hepatobiliary damage results in abnormal liver func-tion and increases the likelihood of gallstones.• Genitourinary damage may result in kidney failure.• Skeletal damage may result in necrosis of the hips and shoulders with a need for joint replacement.• Skin effects may result in chronic ulceration.• Ocular damage in sickle cell anemia is the leading cause of blindness in patients of African ancestry.III. Treatment and Disease Management ▶ Supportive• Management of pain crises remains challenging.• Inadequate intake: Severely restricted diets or diets influenced by such factors as poverty, food faddism, or alcoholism, when the use of alcohol takes prece-dence over food.• Impaired absorption. ▶ Individuals at risk of folate inadequacy• Women of childbearing age.• Pregnant women.• Patients with alcohol dependence.• Individuals with malabsorption disorders, that is, postgastric surgery, celiac disease, and inflammatory bowel disease.• Certain treatment regimens impair the utilization of folate, for example, cancer chemotherapy, rheuma-toid arthritis, and medications, such as methotrexate and dilantin. ▶ Dietary factors: sources• In 1998, the Food and Drug Administration required fortification of cereals, breads, flours, pastas, and other grain products with folate.• Dietary sources can be found in Table 35-4 (Chapter35). ▶ Fetal development• Women with inadequate folic acid intake are at in-creased risk of having a baby with neural tube defects.• Spina bifida (myelomeningocele): a severe condi-tion affecting the formation of the nerves of the spinal cord, and resulting in infant paralysis. Spina bifida is described in Chapter59.SICKLE CELL DISEASE11,12 ▶ (SCD) is a hereditary form of hemolytic anemia, result-ing from a defective hemoglobin molecule. ▶ The name is derived from the crescent or “sickle” shape assumed by the erythrocytes when the defective hemo-globin loses oxygen (Figure 68-2). ▶ It is an autosomal recessive trait and approximately 10% of black Americans have sickle cell trait. Those with sickle cell trait demonstrate few symptoms unless placed under severe stress.FIGURE 68-2 Sickle Cell Disease. Left, diagrammatic drawing of normal red blood cells. Right, sickle shapes of red blood cells of a patient with SCD.Wilkins9781451193114-ch068.indd 1154 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1155 ▶ Implement a comprehensive preventive program to minimize oral infection and control oral disease risk factors. ▶ Avoid long complicated dental appointments by main-taining good oral health with frequent preventive care appointments with the dental hygienist. ▶ Use local anesthesia with low doses of vasoconstrictors to avoid intravascular occlusion of red blood cells.POLYCYTHEMIASPolycythemia implies an increase in the number and con-centration of red blood cells above the normal level. There are two categories of polycythemia: primary and secondary.I. Polycythemia Vera (Primary Polycythemia)14–16 ▶ Cause• Polycythemia vera (PV) is a neoplasm caused by a genetic mutation increasing the sensitivity of bone marrow cells to erythropoietin resulting in an in-creased production of red blood cells.14,15• Blood viscosity increases, affecting oxygen transport to tissues. ▶ Clinical signs and symptoms• Average age at diagnosis is 60.• Increased bleeding risk with spontaneous bleeding of the gingiva.• Bruise easily resulting in submucosal petechiae and hematoma formation.• Risk of fatal and nonfatal blood clot formation lead-ing to heart attack, stroke, and pulmonary embolism.• Migraines.• Vertigo.• Fatigue.• Purplish or red areas on the oral mucosa, gingiva, lips or tongue. ▶ Treatment• Chemotherapy.• Phlebotomy, to reduce the total volume, and par-ticularly the red cell volume, of the blood.• Low-dose aspirin (<100 mg/daily) as an antiplatelet. ▶ Dental hygiene treatment considerations• Thorough review of the medical history due to the increased risk of bleeding, bruising, cerebral vascular accident, and myocardial infarction.• Consult with the hematologist and/or primary care provider about disease management and pa-tient’s blood test results, especially hemoglobin and hematocrit.• Careful attention to oral self-care and preventive maintenance is required to maintain good oral health. ▶ Oxygen is a mainstay of treatment to minimize hypoxia. ▶ Pain relief without depressing breathing. ▶ Avoid substance abuse. ▶ Fever ▶ Antibiotics for infectious diseases to avoid sepsis. ▶ Prevention of sickling• Pharmacologic therapy using a chemotherapy agent (Hydroxyurea) to increase hemoglobin F decreases the permanent formation of sickle cells.• Stem cell transplant may be an option.• Use of prophylactic penicillin to prevent pneumonia and meningococcal sepsis.• Regular blood transfusions.• Gene therapy shows promise in the future.IV. Oral Implications12,13 ▶ Radiographic findings• Coarse trabecular pattern appearing as horizontal rows between teeth (“step-ladder”), with large mar-row spaces.• Osteoporotic changes. ▶ Oral manifestations12,13• Necrosis of the dental pulp.• Osteomyelitis of the mandible.• Enamel hypomineralization.• Overgrowth of the facial bones resulting in protru-sion of the maxilla causing malocclusion.• Pallor of buccal mucosa.• Numb chin syndrome (mental nerve neuropathy).• Facial and dental pain.V. Appointment Management ▶ Thoroughly review the comprehensive medical history. Gather information on the patient’s:• Related complications specific to organ damage and other problems since birth.• Characteristics of pain control (frequency, duration, average number, date of last crisis).• Past and current medical treatment (surgeries, trans-fusions, medications, allergies).• Presence of venous access catheters and joint replacement.• Growth and development issues. ▶ Consultation with the patient’s primary care provider to include the following:• Determine disease control including complete blood count.• Some patient’s require the use of prophylactic antibi-otics. For a patient so highly susceptible to infection, antibiotics may be considered routine, because any form of tissue manipulation creates a bacteremia. ▶ A stress reduction protocol is necessary to prevent pre-cipitation of a sickle cell crisis.Wilkins9781451193114-ch068.indd 1155 07/10/15 11:40 AM 1156 SECTION IX | Patients with Special Needs ▶ Consultation with the primary care provider is neces-sary to determine if neutrophil count is at a safe level. ▶ Antibiotic prophylaxis may be needed.II. Lymphocytopenia17Abnormally low number of lymphocytes in the blood.A. Etiology ▶ Acquired lymphocytopenia may be caused by protein–energy malnutrition, AIDS, chemotherapy, radiation therapy, or infectious disease such as hepatitis, influ-enza, and tuberculosis. ▶ Hereditary lymphocytopenia may be associated with in-herited immunodeficiency disorders.B. Signs and Symptoms ▶ Pallor ▶ Bruising (petechiae) ▶ Mouth ulcersC. Dental Treatment Considerations ▶ The same as for neutropeniaIII. LeukocytosisLeukocytosis is an increase in the number of circulating white blood cells. ▶ Caused by inflammatory and infectious states, trauma, exertion, and other conditions listed in Table 68-2. ▶ The most extreme cause of leukocytosis is leukemia.18• Leukemias are malignant neoplasms of immature white blood cells that multiply uncontrollably.• Cancer cells are located within the circulating blood and in bone marrow; infiltrated into other body tissues and organs such as the spleen and lymph nodes.• Leukemias can be acute such as acute lymphocytic leu-kemia or chronic such as chronic lymphocytic leukemia.• Oral manifestations may include bruising and bleed-ing of the gingiva.PLATELET DISORDERS19Platelets function in the clotting system and disorders in-clude abnormal increases or decreases in platelets or plate-let dysfunction. ▶ When the number of platelets decreases, the risk for bleeding increases.• Risk of bleeding increases when platelet count is be-low 50,000/μL.I. Thrombocytopenia ▶ A lower number of platelets may be caused by decreased production in the bone marrow.• Carefully monitor bleeding and monitor clotting during and following instrumentation.• Provide careful postoperative instructions for early identification of bleeding that does not stop after ap-plying pressure.II. Secondary Polycythemia15 ▶ Secondary polycythemia is also called erythrocytosis, which means an increase in the numbers of red blood cells. ▶ The increased red cell production can result from hy-poxia like that experienced by residents of high alti-tudes, chronic obstructive pulmonary disease, cyanotic heart disease, emphysema, and tobacco smoking. ▶ Bleeding tendencies may be partially controlled with control of gingival irritants.DISORDERS OF WHITE BLOOD CELLS ▶ Disorders of the white blood cells may occur because of a decrease (leukopenia) or an increase (leukocytosis) in cell numbers. ▶ The types of white blood cells are described in Ta-ble68-2 and illustrated in Figure 68-1.I. Neutropenia17A decrease in the total number of neutrophils results when cell production cannot keep pace with the turnover rate or when an accelerated rate of removal of cells occurs, as in certain disease states.A. Etiology ▶ Defects in myeloid cells that may be genetic. ▶ Secondary neutropenia may develop in the following conditions: alcohol abuse, autoimmune disease (e.g., HIV/AIDS), chemotherapy or radiation therapy, folate or vitamin B12 deficiency, infection, or bone marrow transplant.B. Signs and Symptoms ▶ In neutropenias resulting from defects in myeloid cells, the patient may experience oral stomatitis and lymph node enlargement. ▶ Frequent, severe, or unusual infections such as pneumonia.C. Diagnosis ▶ Moderate neutropenia (500–1,000/microliter μL of blood) ▶ Severe neutropenia (<500/μL)• When values drop below 500/μL, even normal mi-crobial flora in the mouth can cause infection.D. Dental Treatment Considerations ▶ Thorough review of the medical history is essential.Wilkins9781451193114-ch068.indd 1156 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1157• History of difficulty in controlling bleeding by usual procedures.• History of bruising easily, with large ecchymoses.• Numerous petechiae.• Marked pallor of the mucous membranes.• Atrophy of the papillae of the tongue.• Persistent sore or painful tongue (glossodynia).• Acute or chronic infections, such as candidiasis, that do not respond to usual treatment.• Severe ulcerations associated with a lack of response to treatment.• Exaggerated gingival response to local irritants, sometimes with characteristics of necrotizing ul-cerative gingivitis (ulceration, necrosis, bleeding, pseudomembrane).II. Types of Disorders of Coagulation20Vascular fragility is increased; petechial and purpuric hem-orrhages appear in the skin or mucous membranes, includ-ing the gingiva.A. Acquired Disorders ▶ Vitamin K deficiency• Vitamin K is essential in the synthesis of prothrom-bin and clotting factors VII, IX, and X.• Food sources of vitamin K may be found in Table35-3. Vitamin K is also produced in the ali-mentary track by intestinal bacteria.• Excessive exposure to antibiotic therapy or pro-longed gastrointestinal disturbances may affect vita-min K production. ▶ Liver disease: Nearly all the clotting factors are pro-duced in the liver. When the liver is not functioning properly, the clotting factors may be altered.• May occur in cirrhosis. ▶ Anticoagulation drugs: Following are the examples of some common medications• Heparin• Coumadin (warfarin)• Aspirin and nonsteroidal anti-inflammatory drugs (NSAIDS).B. Hereditary DisordersAt least 30 hereditary coagulation disorders exist, each resulting from a deficiency or abnormality of a plasma protein. ▶ HemophiliaHemophilias are the oldest known hereditary bleeding disorders caused by low levels or complete absence of a blood protein essential for clotting.• Etiology• Results from mutation or deletion affecting factor VIII or IX in the gene. ▶ Bone marrow depression may be due to drugs, such as hydrochlorothiazide (used to treat high blood pressure), and acetaminophen, infections like HIV and hepatitis, or blood transfusions. ▶ If severe, a platelet transfusion may be necessary.II. Platelet Dysfunction ▶ Acquired platelet dysfunction• Causes of acquired platelet dysfunction include cirrho-sis, systemic lupus erythematosus, and certain drugs. ▶ Hereditary platelet dysfunction• Dysfunction may occur because of a disease such as von Willebrand’s disease (a coagulation disorder).BLEEDING OR COAGULATION DISORDERS ▶ Blood clotting or hemostasis is the body’s mechanism for stopping injured blood vessels from forming clots, which can cause serious health problems. ▶ The three main processes of blood clotting include con-striction of bleeding vessels, activity of platelets, and activity of blood clotting factors. ▶ A history or suspicion of a bleeding problem requires careful evaluation before treatment can be started. ▶ Spontaneous bleeding occurs as small hemorrhages into the skin or mucous membranes and other tissues, and appears as petechiae or purpura. ▶ Bleeding disorders have in common the tendencies to spontaneous bleeding and moderate to excessive bleed-ing following trauma, surgical procedure, or dental hy-giene therapy, including nonsurgical instrumentation.I. Oral Findings Suggestive of Bleeding Disorders ▶ Early signs of systemic conditions frequently appear in the oral soft tissues and clinical examination may iden-tify these changes. ▶ Referral for medical examination may lead to diagnosis and treatment of a serious disease. ▶ In addition, the findings of a laboratory blood exami-nation may provide essential information for safe and effective dental hygiene care. ▶ Oral soft tissue changes in patients with blood diseases are not necessarily exclusive to systemic blood disorders. ▶ It is necessary to recognize change in a previously healthy patient, or an apparently exaggerated response in a patient being examined at an initial appointment. ▶ Findings suggesting a blood disorder include the following:• Gingival bleeding, spontaneously or excessive bleed-ing on gentle probing.Wilkins9781451193114-ch068.indd 1157 07/10/15 11:40 AM 1158 SECTION IX | Patients with Special Needsan appointment or report their most recent INR level to the oral health provider. ▶ Basic tests are listed in Table 68-1 with their ranges or values. ▶ Indications for screening and preappointment tests:• When the patient gives a history of a bleeding problem.• Clinical examination reveals signs of a bleeding disorder.• Patient is being treated with anticoagulation therapy.II. Patient HistoryA. Medical History ReviewThe medical history needs to include information regard-ing the type, severity, medical treatment, medications, and family history of the blood clotting defect. ▶ Question patient specifically regarding bleeding after clinical and surgical procedures, such as tooth extractions.B. Medication ReviewA careful review of a patient’s drug, supplements, and herb use is critical to determine potential oral and physiologic effects. ▶ The primary purpose of the drug and potential side effects needs careful review in a current drug reference guide. ▶ A variety of drugs and herbs may be factors in increased bleeding.• Herbs and supplements associated with increased bleeding are listed in Box 68-2.• It is recommended to discontinue these herbs and supplements 2 weeks prior to receiving invasive sur-gical procedures.21• Hemophilia is an X-linked recessive genetic disease. The defective gene is located on the X chromo-some; thus, the disorder occurs primarily in males.• Treatment• Most patients do well with proper medical man-agement involving the administration of drugs to decrease bleeding or the infusion of platelets or plasma containing factors.• Effects and long-term complications• Bleeding and bruising from minor trauma vary de-pending on the severity of the disease.• Bleeding into the soft tissue of joints (hemarthro-ses) of knees, ankles, and elbows begins in the very young with severe hemophilia.• Hemorrhage into the muscles (intramuscular hemorrhage) is accompanied by pain and limita-tion of motion.• Bleeding from the gingiva is common and more ex-tensive when periodontal infection is more severe.• Management of uncontrolled bleeding• When uncontrolled bleeding is observed, stop dental treatment.• If clotting does not occur within a few minutes, apply digital pressure to area with sterile gauze.• If needed, local hemostatic agents can be applied, such as absorbable gelatin sponge. Absorbs for 3–5 days.• Medical attention required if unsuccessful in stopping bleeding.DENTAL HYGIENE CARE PLAN ▶ Although prevention and control of bleeding are the main issues when treating a patient with a blood clot-ting defect, other factors require attention. ▶ Patients with bleeding disorders have many emotional stresses related to the disease, its treatment, and medi-cal expenses. ▶ As a result of internal and cerebral hemorrhages, a few patients may have a multitude of physical or cognitive disabilities. ▶ Because of the fear of bleeding, patients may neglect toothbrushing and flossing; doing so can lead to in-creased dental biofilm accumulation and inflammation. ▶ Suggestions for appointments from Chapter 57 may be useful for the patient with disability related to hemar-throses and orthopedic treatment.I. Preparation for Clinical Appointment ▶ Certain blood tests may be needed on the same day as treatment.• For example, the patient taking coagulants is required to have a PT (prothrombin time) within 24 hours of Alfalfa Cat’s claw GarlicAllspice Chamomile GinkgoAngelica Chondroitin GingerAnise Coenzyme Q10 GinsengBilberry Cranberry GlucosamineBlackhawk Dong quai GuggulBogbean Evening primrose Horse chestnutBoldo Fenugreek PoplarBucho Feverfew Omega 3Capsicum Flax Vitamin EFish oil White willowAdapted from Alberto PL. Alternative medicine for the dental profes-sional. 2009;23(1):20–24. Herbs and Supplements Associated With Increased BleedingBOX 68-2Wilkins9781451193114-ch068.indd 1158 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1159 ▶ Preliminary tissue conditioning can help prevent severe bleeding during instrumentation. Teach daily personal biofilm removal at the initial appointment and rein-force at each session. ▶ Use a preprocedural chlorhexidine mouthrinse to reduce the bacterial load prior to beginning examination or treatment. ▶ Radiographic imaging: Films/sensor (infection control barriers) can cut and press on the mucous membranes. Care in placement is exercised to avoid bleeding and/or a hematoma. ▶ Encourage each patient to improve and maintain good oral health. Spontaneous oral bleeding problems can be partially controlled by the elimination of oral infections.23• Meticulous dental biofilm removal is practiced and repeated. A soft toothbrush is indicated. If patient indicates using a power brush, proper demonstration of technique is necessary.• Teach flossing carefully and correctly to prevent cut-ting the gingiva and inducing proximal bleeding.• Patients with limited manual dexterity can benefit from adaptive oral hygiene aids described in Chapter 57. ▶ Select age-appropriate preventive measures based on risk assessment including fluoride treatments, remin-eralizing agents, sealants, biofilm control, and profes-sional dental supervision. ▶ A nutritional assessment and counseling is recommended for caries control and periodontal health to stress choos-ing foods that provide a healthy, well-balanced diet. ▶ Plan non-surgical periodontal therapy in sextants or quadrants. ▶ Local anesthesia administration: infiltration and intra-papillary injections do not require administration of clotting factors to prevent bleeding, but blocks like the inferior alveolar injection require clotting factor.23 ▶ Postoperative: Monitor to ensure bleeding has stopped postoperatively.• Never recommend aspirin or NSAIDS for a patient with a bleeding disorder.• Bleeding tendency is greatly increased by a drug-induced platelet dysfunction caused by aspirin.• Ask the patient to call if bleeding occurs within first 24–48 hours and does not stop after applying pressure.23 ▶ Impressions: Beading the rims of the trays protects the mucosa from pressure and damage from a hard, possibly rough surface, as described in Chapter 14. ▶ Evacuation: High-vacuum suction tips may be sharp. Caution in the use of suction is necessary to prevent pulling the sublingual or other mucosal tissues into the suction tip and causing hematomas. ▶ Stress reduction protocol: Prevention or reduction of stress begins before the appointment, is continued ▶ Patients taking either Coumadin (for prevention of recurrent thrombosis), heparin (for short-term use fol-lowing a total joint replacement procedure), or medi-cation for long-term anticoagulation (such as aspirin) may need special consultation.• The most common side effect of both warfarin and heparin is hemorrhage.• Hemorrhages may present as gingival bleeding or submucosal bleeding with hematoma formation. ▶ Cancer chemotherapeutic agents may secondarily in-duce profound thrombocytopenia (<20,000 mm3) or neutropenia (<500 mm3). ▶ Antithrombotic agents such as aspirin and clopidogrel alter the ability of platelets to stick or clump together and form a clot.C. Dental History ReviewDiscuss previous dental care and perceived treatment needs when developing the current care plan with the patient.D. Risk AssessmentThe patient with a bleeding disorder may be at high risk for dental caries and periodontal disease and the appropriate strategies to manage the risk are employed.III. Consultation with Physician/Hematologist ▶ Consultation with the primary care provider/hematologist is necessary to obtain complete and accurate information. ▶ The risk for intravascular clot formation is greater than the risk for hemorrhage. ▶ A joint policy advising healthcare providers who per-form invasive or surgical procedures to contact the cardiologist and discuss patient management before dis-continuing antiplatelet drugs has been developed by:22 • The American Dental Association• The American Heart Association• The American College of Cardiology• The Society for Cardiovascular Angiography and Interventions• The American College of Surgeons. ▶ Consult primary care provider/hematologist to deter-mine whether premedication is required during dental procedures to prevent infection in joint prostheses and/or indwelling catheter.22 ▶ Many procedures require factor replacement therapy just prior to the dental appointment.23 ▶ Request reports of current blood tests.IV. Treatment ▶ Prevention of gingival infection and dental caries is an es-sential aspect of care for patients with a bleeding disorder.Wilkins9781451193114-ch068.indd 1159 07/10/15 11:40 AM 1160 SECTION IX | Patients with Special NeedsJust as Dena, the dental hygienist, begins to probe for re-cording the gingival examination for Mr. Bennett, a new patient in the practice, the receptionist interrupts to give Dena a medical clearance form that has been faxed from the patient’s physician. As Dena reviews the information, she understands that the patient has a blood disorder but is unclear as to its extent from the laboratory values in the report. She briefly questions Mr. Bennett about any medical tests and he indicates that he was in the hospital 4 days the previous month.As Dena continues the probing, she notices consider-able bleeding with oozing around the gingival margins.Questions for Consideration1. What action, if any, does Dena need to take to en-sure she is performing beneficently on behalf of this patient?2. It appears that Mr. Bennett has not given sufficient information about his medical condition prior to this continuing care appointment. What obligation does a patient have to update the medical history at each appointment? And what obligation does the profes-sional person have to help the patient understand this obligation?3. While Dena quietly acknowledges to herself that she used to know the information about bleeding condi-tions, she is currently uncertain of the meaning of the laboratory values in this patient’s report. Ethically, how can this realization be assessed? What is the immedi-ate need? What can be done to prevent such a situa-tion from occurring in the future?EVERYDAY ETHICSExample Documentation: Patient with a Blood DisorderS – A 70-year-old, white man presents for a new patient exam. He reports having a stroke a year ago and cur-rently taking Coumadin (warfarin). Patient states that his INR last week was 2.8.O – Oral cancer examination: all tissues appear normal. Full-mouth series of radiographs and caries examination re-veal no new caries. Periodontal Examination: generalized 4–5 mm pocket depths with localized bleeding on prob-ing. Plaque score: 50%.A – Increased risk for oral bleeding following dental hygiene treatment.P – Disclosed plaque biofilm and reviewed technique for oral hygiene aids. Stressed need for meticulous oral self-care to prevent infection and associated bleeding. Nonsurgi-cal periodontal therapy completed maxillary and man-dibular right quadrants with 2 carpules 2% xylocaine with 1:100,000 epinephrine for an inferior IA, LB, PSA, and palatal injections. Areas monitored to verify bleeding had stopped within 5 minutes following completion of instrumentation. Postoperation instructions provided to contact the office should he be unable to stop bleeding.Next appointment: Complete NSPT with local anesthesia and apply fluoride varnish.Signed: _____________________________________, RDHDate: _______________________________________BOX 68-3throughout the treatment, and followed through into the postoperative period, when necessary. ▶ Frequency of continuing care: Frequent appointments can aid in keeping the oral tissues in an optimum state of health and help to prevent the need for complex or lengthy dental appointments.DOCUMENTATIONDocumentation in the permanent record for each appoint-ment with a blood disorder includes a minimum of the fol-lowing factors: ▶ Review or update medical history thoroughly. Docu-ment laboratory findings. ▶ Document extra- and intraoral examinations findings. Use written description and an intraoral picture when possible. ▶ Note treatment planning considerations such as shorter appointments, stress reduction protocols that need to be implemented, and oral hygiene considerations. ▶ Note consultations with other health professionals in-volved in the patient’s care. ▶ Reference any difficulties with bleeding control during appointment. ▶ A sample progress notes is provided in Box 68-3.Wilkins9781451193114-ch068.indd 1160 07/10/15 11:40 AM CHAPTER 68 | The Patient with a Blood Disorder 1161gov/factsheets/Folate-HealthProfessional/. Accessed Janu-ary 19, 2014.11. Bunn H. Sickle cell disease. In: Bunn H, Aster JC, eds. Pathophysiology of Blood Disorders. New York, NY: McGraw-Hill; 2011:chap 9.12. Sheth S, Licursi M, Bhatia M. Sickle cell disease: time for a closer look at treatment options? Br J Haematol. 2013;162(4):455–464.13. Adeyemo TA, Adeyemo WL, Adediran A, et al. Orofa-cial manifestations of hematological disorders: anemia and hemostatic disorders. Indian J Dent Res. 2011;22: 454–461.14. Squizzato A, Romualdi E, Passamonti F, et al. Antiplatelet drugs for polycythaemia vera and essential thrombocythae-mia. Cochrane Database Syst Rev 2013;(4):CD006503.15. College of Dental Hygienists of Ontario, CDHO Advi-sory Polycythemia. 2012. http://www.cdho.org/Advisories/CDHO_Advisory_Polycythemia.pdf. Accessed January 20, 2014.16. National Cancer Institute. PDQ¯ Chronic Myeloproliferative Disorders Treatment. Bethesda, MD: National Cancer Insti-tute. http://cancer.gov/cancertopics/pdq/treatment/myelo-proliferative/HealthProfessional. Accessed January 20,2014.17. Territo M. Overview of white blood cell disorders. The Merck Manual—Professional Edition. http://www.merckman uals.com/home/blood-disorders/white-blood-cell-disorders/overview-of-white-blood-cell-disorders. Accessed June 29, 2015.18. Rytting ME. Chronic myelogenous leukemia. The Merck Manual—Professional Edition. http://www.merckmanuals .com/professional/hematology-and-oncology/leukemias/chronic-myelogenous-leukemia-cml. Accessed June 29, 2015.19. Kuter DJ. Overview of platelet disorders. The Merck Man-ual—Professional Edition. http://www.merckmanuals.com/professional/hematology_and_oncology/thrombocytope nia_and_platelet_dysfunction/overview_of_platelet_disor ders.html. Accessed January 20, 2014.20. Moake JL. Hemophilia. The Merck Manual—Professional Edition. http://www.merckmanuals.com/professional/hema tology_and_oncology/coagulation_disorders/hemophilia .html. Accessed January 25, 2014.21. Andrews L, Spolarich AE. An examination of the bleed-ing complications associated with herbal supplements, antiplatelet and anticoagulant medications. J Dent Educ. 2007;81(3):2–14.22. American Academy of Orthopaedic Surgeons, American Dental Association. Prevention of Orthopaedic Implant Infec-tion in Patients Undergoing Dental Procedures: Evidence-Based Guideline and Evidence Report. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2012. http://www.aaos.org/Research/guidelines/PUDP/PUDP_guideline.pdf. Ac-cessed January 25, 2014.23. Dental Committee, Word Federation of Hemophilia. Guide-lines for Dental Treatment of Patients with Inherited Bleeding Disorders. 2006. http://www1.wfh.org/publication/files/pdf-1190.pdf. Accessed January 25, 2014.References1. Mesher AL. Blood. In: Mescher AL, ed. Junqueira’s Basic Histology: Text & Atlas. 13 ed. New York, NY: McGraw-Hill; 2013.2. Introduction to anemia and red cell disorders. In: Bunn H, Aster JC, eds. Pathophysiology of Blood Disorders. New York, NY: McGraw-Hill; 2011. http://accessmedicine.mhmedi-cal.com/content.aspx?bookid=676&Sectionid=44827768. Accessed April 25, 2014.3. Damon LE, Andreadis C. Blood disorders. In: Papadakis MA, McPhee SJ, Rabow MW, eds. CURRENT Medical Diagnosis & Treatment 2014. New York, NY: McGraw-Hill; 2014:chap 13.4. Muncie HL Jr, Campbell J. Alpha and beta thalassemia. Am Fam Physician. 2009 15;80(4):339–344.5. Goddard AF, James MW, McIntyre AS, et al. Guidelines for the management of iron deficiency anaemia. Gut. 2011;60(10):1309–1316.6. Office of Dietary Supplements. Iron: Dietary Supplement Fact Sheet. National Institutes of Health. http://ods.od.nih.gov/factsheets/Iron-HealthProfessional/. Accessed January 19, 2014.7. Andres E, Serraj K. Optimal management of pernicious anemia. J Blood Med. 2012;3:97–103.8. Office of Dietary Supplements. Vitamin B12: Dietary Supple-ment Fact Sheet. National Institutes of Health. http://ods.od.nih.gov/factsheets/VitaminB12-HealthProfessional/. Accessed January 19, 2014.9. Medical Services Commission. Cobalamin (vitamin B12) De-ficiency—Investigation and Management. Victoria, BC: British Columbia Medical Services Commission; 2012. http://www .guideline.gov/content.aspx?id=38881. Accessed Janu-ary19, 2014.10. Office of Dietary Supplements. Folate: Dietary Supplement Fact Sheet. National Institutes of Health. http://ods.od.nih. ▷ Meticulous oral hygiene techniques to practice daily: toothbrushing, flossing, and other appropriate oral hy-giene aids. ▷ How to self-evaluate the oral cavity for deviations from normal. Watching for changes in size, shape, color, and contacting oral health professional when lesions last longer than 2 weeks. ▷ Selection of noncariogenic foods to prevent caries, and knowledge about the diet’s relationship to health. ▷ Avoid use of salicylates (aspirin). ▷ Importance of informing the dental hygienist of any changes to the medical history, including drugs, herbs, supplements, and hospitalizations and providing recent laboratory values before beginning treatment.Factors To Teach The PatientWilkins9781451193114-ch068.indd 1161 07/10/15 11:40 AM 1162 SECTION IX | Patients with Special NeedsENHANCE YOUR UNDERSTANDINGDIGITAL CONNECTIONS(see the inside front cover for access information)• Audio glossary• Quiz bankSUPPORT FOR LEARNING(available separately; visit lww.com)• Active Learning Workbook for Clinical Practice of the Dental Hygienist, 12th EditionINDIVIDUALIZED REVIEW(available separately; visit lww.com)• Adaptive quizzing with prepU for Wilkins’ Clinical Practice of the Dental HygienistWilkins9781451193114-ch068.indd 1162 07/10/15 11:40 AM

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