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Hematologic and Bleeding

Disease
Karla Monica Wijaya
Eva Gracia
Raden Aqsa Aditya

Red blood
disorders

Polycythemia Vera

Red Blood Cell
Disorders

Anemia
Hemoglobonopathies

Polycythemia Vera Red Blood Cell Disorders Anemia Hemoglobinopathies .

. thrombosis. Vertigo Gastrointestinal pain Headache Paraesthesias. and increased RBC mass. Clinical Manifestations Usually asymptomatic but when symptomatic : Pruritis.Polycythemia Vera is a chronic myeloproliferative disease characterized by a predominant proliferation of the erythroid cell line and primary bone marrow dysfunction that result in hemorrhage.

cont… Polycythemia Vera Oral Manifestations • Erythema of mucosa • Glossitis • Erythematous and edematous gingiva • Spontaneous gingival bleeding Oral Health Considerations Control of hemorrhage after dental surgery .

Polycythemia Vera Red Blood Cell Disorders Anemia Hemoglobinopathies .

Anemia Anemia Defined as reduction in oxygencarrying capacity of the blood Associated with decreased number of ciculating RBCs Abnormality in the Hb contained within RBCs Anemia may results from 1. pernicious anemia. Increased rate of destruction of circulating RBCs . Decreased production of RBCs (iron deficiency. Blood loss 3. folate deficiency) 2.

Types of Anemia Iron Deficiency Anemia Folate Deficiency Anemia and Pernicous Anemia Hemolytic Anemia Sickle Cell Anemia Aplastic Anemia .

Oral candidiasis .Glossitis .Atrophy of fungiform and filiform papillae . poor iron intake.RAS .Stomatitis .Angular cheilitis .Iron Deficiency Anemia • Microcytic anemia that can be caused by excessive blood loss.Pale oral mucosa . or increased demand for iron. • May occur with menstruation or bleeding from gastrointestinal tract Treatment Planning Modifications Usually none indicated Oral Manifestations .Erythematous mucositis . poor iron absorption.

lips.Burning sensation in tongue. buccal mucosa .Loss of papillae from tongue .Folate Deficiency Anemia and Pernicious Anemia Result from lack of vitamin B12 and folic acid which are needed for RBC formation and growth within bone marrow Treatment Planning Modifications Usually none indicated Oral Manifestations .Dysphagia .Angular cheilitis .Tongue and mucosa may be smooth or patchy areas of erythema .

Hemolytic Anemia Result in anemia if the bone marrow is not able to replenish adequately the prematurely destroyed RBCs Clinical Manifestations Acute back pain Fatigue Loss of stamina Breathlessness Tachycardia Jaundice Hemoglobinuria .

Cont… Hemolytic Anemia Oral Manifestations Pallor or jaundice of oral mucosa Paresthesia of mucosa Hyperplastic marrow spaces in the mandible. maxilla. or that contain aspirin or acetaminophen Avoid aspirin Avoid sulfa drugs and chloramphenicol Perform only urgent dental procedures . and facial bones Dental Management Modifications Avoid drugs such as certain antibiotics.

Aplastic Anemia Rare blood dyscrasia in which peripheral blood pancytopenia results from reduced or absent blood cell production in the bone marrow and normal hematopoietic tissue in the bone marrow has been replaced by fatty marrow Clinical Manifestations Fatigue Malaise Chest pain Shortness of breath Sudden onset of bleeding caused by thrombocytopenia .

Aplastic Anemia Oral Manifestations Hemorrhage Candidiasis Viral infection Petechiae Spontaneous gingival bleeding Gingival hyperplasia from cyclosporine and herpetic lesion Dental Management Modification Attention to details of oral hygiene and hand washing and avoidance of minor injuries .

Polycythemia Vera Red Blood Cell Disorders Anemia Hemoglobinopathies .

or stability Sickle Cell Anemia Hemoglobinopat hies Thalassemia . production rate.Hemoglobinopathies Point mutations or deletion in the globin genes cause changes in the amino acids that make up the globin protein Results in abnormal forms of hemoglobin : its behaviour.

reduced their plasticity and lifetime from the Clinical Manifestations normal 120 days average down to 14 days Invasive infections Painful episodes Acute chest syndrome Cerebrovascular accidental strokes Aplastic crises leading to severe anemia Chronic leg ulcers Hematuria . presenting sickle cell shape.Sickle Cell Anemia Erythrocytes have their normal biconcave discoid shape distorted.

Cont… Sickle Cell Anemia .

increased overjet Pallor of the oral mucosa Delayed eruption of the teeth Gnatopathy Dental Management Modifications Antibiotic prophylaxis (amoxicillin) before dental procedures such as dental extractions. calcified canals. Avoidance of use of aspirin . enamel hypomineralization. treatment under general anaesthesia.Cont… Sickle Cell Anemia Oral manifestations Radiographic findings include a stepladder trabecullae pattern. increased overbite.

Cont… Sickle Cell Anemia .

Thalassemia Abnormal formation of hemoglobin that result in improper oxygen transport and destruction of red blood cells. Reduced RBCs. lowered hemoglobin. Clinical Manifestation Seen in early childhood Fatigue Weakness Pale skin Jaundice . and defects in α and β chains of hemoglobin.

Thalassemia
Oral Manifestations
o Spiky-shaped and short roots
o Taurodontism
o Attenuated lamina dura
o Enlarged bone marrow spaces
o Small maxillary sinuses
o Absence of inferior alveolar canal
o Thin cortex of the mandible
o Class II skeletal (short mandible, reduced posterior
facial height, increased anterior facial proportion)
o Higher dental caries with low saliva concentrations of
IgA and phosphorus

white blood
disorders

White Blood Cell
Disorders
Quantitative
Leukocyte
Disorders

Qualitative
Leukocyte
Disorders

Leukemia

Multiple
Myeloma

Lymphoma

White Blood Cell
Disorders
Quantitative
Leukocyte
Disorders

Qualitative
Leukocyte
Disorders

Leukemia

Multiple
Myeloma

Lymphoma

Granulocytosis Quantitative Leukocyte Disorders Agranulocytosis (Neutropenia/Granuloc ytopenia) Cyclic Neutropenia Chronic Neutropenia .

Granulocytosis Quantitative Leukocyte Disorders Agranulocytosis (Neutropenia/Granuloc ytopenia) Cyclic Neutropenia Chronic Neutropenia .

myeloproliferati veParasitic disorders diseases. tissue destruction. certain allergic diseases.Granulocytosis is an abnormaly large number of granulocytes in the blood Granulocytosi s Basophilia High amount of basophils Eosinophili a High amount of eosinophils Neutrophili a High amount of neutrophils Chronic hypersensitivity . . inflammation toxic. chronic skin diseases Infection.

Cont… Granulocytosis Oral Manifestations Necrotic ulcers with a white or greyish surface without signs of inflammation .

Granulocytosis Quantitative Leukocyte Disorders Agranulocytosis (Neutropenia/Granuloc ytopenia) Cyclic Neutropenia Chronic Neutropenia .

Agranulocytosis (Neutropenia/Granulocytopenia) is a condition that has a reduced quantity of absolute neutrophil count (ANC) of circulating neutrophils in blood Clinical Manifestations Fever Rigor and sore throats Oral manifestations Oral ulcerations in palate and tongue Periodontitis Necrotic tissue Alveolar bone loss Tooth mobility Bleeding on probing .

root planning and extraction under corticosteroid and antibiotic cover Dental Management Modification  Avoid antibiotic that increase incidence agranulocytosis (macrolides. cephalosporin)  During of low blood count. provide emergency care only  Treatment should include use of antimicrobial agents and supportive therapy for oral lesions  Susceptible to infection .Agranulocytosis (Neutropenia/Granulocytopenia) Dental treatment consisted of scaling. penicillins.

Granulocytosis Quantitative Leukocyte Disorders Agranulocytosis (Neutropenia/Granuloc ytopenia) Cyclic Neutropenia Chronic Neutropenia .

Cyclic Neutropenia is a rare disease characterized by cyclical depression of the peripheral blood polymorphonuclear leukocyte (PMNL) count at 21-day intervals Manifest initially in infant or childhood Clinical Manifestation Infection Fever Lymphadenopathy .

Cont… Cyclic Neutropenia Oral Manifestation Periodontal disease Oral infection Oral ulceration similar to that of aphtous stomatitis Stomatitis Gingivitis Early onset of periodontitis in childhood unusual form of ulcerative gingivitis associated with gingival ecchymoses .

Cyclic Neutropenia Dental Management Modification Susceptible to infection Antibiotics should be given to prevent infection Modification care not required when WBC count is normal If WBC count is deppressed. antibiotics should be provided to prevent postoperative infection .

Granulocytosis Quantitative Leukocyte Disorders Agranulocytosis (Neutropenia/Granuloc ytopenia) Cyclic Neutropenia Chronic Neutropenia .

early onset in childhood Early tooth loss Oral Manifestations Low-grade fever Sore mouth Odynophagia Gingival pain and swelling Skin abscesses Recurrent sinusitis and otitis Symptoms of pneumonia (eg.Chronic Neutropenia is defined as a low ANC for more than 6 months Clinical Manifestations Life-threatening bacterial infections Recurrent gingivitis Severe periodontitis. cough. dyspnea) Perirectal pain and irritation .

White Blood Cell Disorders Quantitative Leukocyte Disorders Qualitative Leukocyte Disorders Leukemia Multiple Myeloma Lymphoma .

and defective neutrophil function Clinical Manifestations Oculotaneous albinism Photophobia Neurologic features (peripheral neuropathy) Recurrent infections Enterocolitis . deficient platelet dense bodies. neutropenia.Chronic Qualitative Leukocyte Disorders Chediak-Higash Syndromec Neutropenia is a rare autosomal recessive immunodeficiency disorder.

and Aspergillus Early onset periodontitis Extensive loss of alveolar bone Tooth exfoliation Dental Management Modification Avoid intramuscular injection Bring sunglasses to avoid sensitive to the bright operatory lights . Candida.ChroniQualitative Leukocyte Disorders Chediak-Higash Syndromec Neutropenia Oral Manifestations Serious periodontal destruction with acute inflamed gingiva and ulcers Sinus and oral infection caused by β-hemolytic Streptococcus. gram-negative organism. S. aureus.

White Blood Cell Disorders Quantitative Leukocyte Disorders Qualitative Leukocyte Disorders Leukemia Multiple Myeloma Lymphoma .

Leukemia Leukemia is cancer of the WBCs that affects the bone marrow and circulating blood. involves exponential proliferation of a clonal myeloid or lymphoid cell Acute Leukemia Leukemia Chronic Leukemia Acute Lymphocyti c Leukemia Acute Myelogeno us Leukemia Chronic Lymphocyti c Leukemia Chronic Myelogeno us Leukemia .

spleen.Acute Lymphocytic Leukemia is the result of uncontrolled monoclonal proliferation of immature lymphoid cells in the bone marrow anf peripheral blood. small hemorrhage in skin and mucous membrane) Poor healing Infection after surgical procedures . bleeding. dyspnea. liver. or CNS Clinical manifestations Petechiae skin Posterior palate hemorrhage Gingival bleeding Gingival infiltration Gingival ulceration as a result of infection by normal oral flora Excessive bleeding Anemia (malaise. pallor. Expand to lymph nodes.

Acute Lymphocytic Leukemia Dental Management Modification Prechemotherapy dental assessment is needed All needed dental procedures is have to be done before chemotherapy .

complications of chemotherapy Fatigue Easy bruising Bone pain Flu-like symptoms for 4 to 6 weeks Oral ulcerations Fever . which demostrate uncontrolled proliferation in the bone marrow space and appea in the peripheral blood. dyspnea. bleeding.Acute Myelogenous Leukemia is a neoplasm of myeloid (immature) WBCs. Clinical Manifestations Excessive bleeding Anemia (malaise. pallor. small hemorrhage in skin and mucous membrane) Poor healing Infection after surgical procedures Hemorrhage and infection.

Chronic Lymphocytic Leukemia is a neoplasm of mature clonal CD5+ B lymphocytes Clinical Manifestations Poor wound healing Risk for infection Fatigue Anorexia Weight loss Enlarged spleen Lymphadenopathy Decreased serum immunoglobulin levels Fever Oral Manifestations Generalized lymphadenopathy Pallor of the mucosa Hypoglobulinemia .

Chronic Myelogenous Leukemia is a neoplasm of mature myeloid WBCs .

treatment. bleeding. and antifungals provided during chemotherapy to prevent opportunistic oral infection • Chlorhexidine rinse rinses to manage mucositis • Eliminate infections before chemotherapy • Extractions should be performed at least 10 days before initiation of chemotherapy • Use prophylactic antibiotic if WBC count less than .Dental Modification for Leukemia • Referral for medical diagnosis. and infection • Antibiotics. antivirals. and consultation • Complete blood count to determine risk for anemia.

White Blood Cell Disorders Quantitative Leukocyte Disorders Qualitative Leukocyte Disorders Leukemia Multiple Myeloma Lymphoma .

Hodgkin’s Lymphoma Lymphoma NonHodgkin’s Lymphoma Burkit’s Lymphoma .

. histiocytes. – The presence of the malignant cell (the ReedSternberg cell). – Inflammatory cellular infiltrate (lymphocytes.LYMPHOMA Hodgkin’s Lymphoma • Etiology: unknown. plasma cells and fibroblasts). but both genetic and environmental factors play a role in pathogenesis of HL. • Morphologic feature: – Effacement of the involved lymph node with destruction of its normal architecture.

Treatment • Systemic symptoms: night sweats. fever and weight loss. often in the neck. • Mediastinal lymph node involvement is common. • Radiation and chemotherapy • Radiation that involve bilateral cervical nodes have the potential to result in damage to salivary .• First sign: an asymptomatic enlargement of a supradiaphragmatic lymph node. cough and dyspnea. • Patients may complain of chest pain.

topical fluoride varnish. • Patients who receive radiation in fields involving the cervical nodes will have their submandibular and sublingual salivary glands in the field and are at risk for temporary. gel or fluoride toothpaste 1000 ppm can be used. • Risk of osteoradionecrosis is very low  low radiation doses & often exclusion of maxilla and mandible from the fields. . • If the patient’s mouth appear to dry or caries rate appears elevated.Oral Manifestation HL rarely presents as an extranodal mass in the Oral Health Consideration head and neck region. and occasionally permanent xerostomia.

Hodgkin’s Lymphoma Lymphoma NonHodgkin’s Lymphoma Burkit’s Lymphoma .

bone marrow. chills. night sweats and weight loss. thyroid and CNS. • Clinical Manifestation: – Lymphadenopathy and sometimes extranodal involvement of the GI tract. skin. immunotherapy or radioimmunotherapy .LYMPHOMA Non-Hodgkin’s Lymphoma • Associated with chronic inflammatory disease and immune suppresion. – Systemic symptoms: fever. • Treatment: – Early stage: radiation therapy – Stage III&IV: chemotherapy. sinuses.

blunted.Oral Manifestation • Nonhealing ulceration with ill-defined borders in the surrounding mucosa. include •• Acute complications of chemotherapy mucositis. delayed and ectopic eruptions  . viral and bacterial infections and hemorraghic lesions related to bone marrow suppression. • Erosive mucosal lesions resembling a Oral Health Consideration vesiculobullous disease. Paresthesia of oral mucosal tissue. • Benign-appearing gingival lesions. • Short. • Enamel discoloration and root malformations  long-term survivors of NHL treated with chemotherapy. tapered and V-shaped root malformations.

Hodgkin’s Lymphoma Lymphoma NonHodgkin’s Lymphoma Burkit’s Lymphoma .

breast and other lymphomas in children in the US. ovaries.LYMPHOMA Burkitt’s Lymphoma Sporadic • 15-30% EBVnon-cleaved cell • AEndemic highly aggressive. small. • Occurs mainly in patients infected with HIV but also occurs in organ transplant recipients and individual with congenital immunodeficien . 40% ofof BL • Male:female = 2:1 • EBV associated • Involving primarily the bones of the jaw and other facial bones. • Accounts up to African children cy-associated • (4-7years Three clinical variants • Often EBV old). associated. • Occuring in associated Immunodeficien lymphoma. as well as the kidneys. • Most common site of involvement: abdomen • May have malignant pleural effusions or ascites and involvement of the lymph nodes. GI tract.

surgical debulking and Oral Manifestation palliative low-dose radiation therapy. • Jaw tumors may result in tooth mobility and pain. destruction of cortex around tooth crypts. loss of teeth lamina dura. intraoral swelling of the mandible and maxilla and anterior open bite.Treatment Chemotherapy. enalargement of tooth follicles. • Radiographic features: resorption of alveolar bone. .

anemia and hypercalcemia. – Tooth mobility. weight loss. – Paresthesia of the inferior alveolar and mental nerve. abnormal paraprotein and complication of bone disease with skeletal destruction. bone pain and recurrent infections.MULTIPLE MYELOMA • Plasma cell neoplasm that is characterized by a bone marrow plasmacytosis. • Clinical Manifestation: fatigue. weakness. . renal insufficiency or failure. – Pain. • Oral Manifestation: – Soft tissue masses that are extramedullary plasmablastic tumors of the jaws.

surgery and analgesic medication to manage focal bone lytic lesion. • Management of ONJ: anstiseptic mouthrinses and antibiotics and avoidance of unnecessary invasive dental proceudres. • Patients with MM can manifest unique hemostatic disorders. • Prevention of hemorrhage  consulaltion with the patient’s . • Radiation therapy. • Intravenous infusions of biphosphonate have reduced the Oral Health Consideration skeletal complications. • Complication of long-term intravenous biphosphonate therapy  osteonecrosis of the jaw (ONJ). predisposing the patient of hemorrhage.Treatment • Chemotherapy improves the likelihood of a complete respons and prolongs disease-free survival and over all survival.

Bleeding and clothing disorders .

MECHANISM OF HEMOSTASIS .

hematomas or jaundice. . ecchymoses. spider angiomas.Clinical Manifestation CLINICAL AND LABORATORY FINDINGS • Mild disease: no clinical signs • Severe: definite stigmata • When skin and mucosa involved: petechia.

Factor assays Test of capillary fragility • To further identify factor deficiencies and • their The tourniquet test forspecific capillary fragility is level of severity.CLINICAL LABORATORY TESTS To Evaluate the Status of Other Aspect of Hemostasis Prothrombin time (PT) • Evaluates the extrinsic coagulation system and measures the presence or absence of clotting FI. • aPTT is prolonged only when the factor levels in Fibrin degradation products the intrinsic and common pathways < 30% (FDPs) • To evaluate the presence of the Ddimer of fibrinogen and/or fibrin above normal levels. . partial thromboplastin Activated time (aPTT) To Evaluate Primary Hemostasis Platelet Count Bleeding Time • To evaluate the intrinsic cascade and measure the functional levels of F VIII. activity levels useful for identifying disorders of vascular of factors can be measured. Thrombin time (TT) • To test the ability to form the initial clot from fibrinogen.II.and X.V.IX.XI and XII.VII.

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Classification of Bleeding Disorders Vessel Wall Disorders Platelet Disorders • Congenital Platelet Defects • Acquired Platelet Defects Coagulation Disorders • Congenital • Acquired .

• Patient may show skin bleeding or easy bruising. • Purpura senilis: irregularly shaped purpuric areas on arms and hands because of tears in small blood . • Hemorrhage can occur in muscles.Scurvy Wall Disorders Cushing’s Vessel • Vitamin C deficiency. • Clinical signs: petechial hemorrhage at the hair follicles and purpura on the back of the lower extrimities. • Defect in collagen synthesis. and syndrome • Resulting from excessive exogenous or endogenous corticosteroid intake or production  general protein wasting and atrophy of supporting connective tissue around blood vessels. nail beds. joints.

tongue and palate that may bleed upon manipulation during . • Resulting in fragile skin blood vessels and easy bruising. • Perioral and intraoral angiomatous nodules are common with progressive disease involving areas of the lips. • Characterized by hyperelasticity of the skin and hypermobile joints.Ehlers-Danlos syndrome • Autosomal dominant inherited disorder of connective tissue matrix. frequent episodes of nasal and gastrointestinal bleeding and associated brain and pulmonary lesions. syndrome • Group of autosomal dominant disorders with abnormal telangiectatic capillaries.

Platelet Disorders • Thrombocytopenia: platelet quantity is reduced and caused by one of three mechanism: – Decreased production in the bone marrow – Increased sequestration in the spleen – Accelerated destruction • Thrombocytopathies: qualitative platelet disorders. may result from defects in any of the three critical platelet reactions: adhesion. . or granule release. aggregation.

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Congenital Coagulopathi es Coagulapathi es Anticoagulant -related Coagulopathi es Diseaserelated Coagulopathi es .

Congenital Coagulopathi es Coagulapathi es Anticoagulant -related Coagulopathi es Diseaserelated Coagulopathi es .

Congenital Coagulopathies • Deficiency in coagulation factors • vWD disease (von Willebrand disease) .

• Hemophilia A : deficiency of factor VIII • Hemophilia B (Christmas disease) : deficiency of factor IX. • Hemophilia C : deficiency of factor XI. • Deficiency of factor VIII and IX: bleeding of multiple sites. frequently manifested in the mouth as gingival and post-extraction hemorrhages .Hemophilia • X-linked hereditary disorder.

vWD disease (von Willebrand disease) • vWD is classified into three primary categories – Type 1  Partial quantitative deficiency – Type 2  Qualitative defect • • • • Type Type Type Type 2A 2B 2M 2N – Type 3  virtually complete deficiency of vWD .

Anticoagulant-related Coagulopathies • Heparin Heparin is a potent anticoagulant that binds with antithrombin III to significantly inhibit activation of Fs IX. X. and XI • Coumarin They slow thrombin production and clot formation by blocking the action of vitamin K .

Abnormal vitamin K–dependent factor and fibrinogen molecules have been encountered. VII. important factors and inhibitors of the clotting and the fibrinolytic systems are markedly reduced. IX.Disease-related Coagulopathies Liver disease Owing to impaired protein synthesis. and X. Vitamin K deficiency Vitamin K deficiency is assosiated with the production of poorly functioning vitamin K dependent Fs II. .

Disease-related Coagulopathies Disease-related Coagulopathies Disseminated intravesel coagulation DIC is triggered by potent stimuli that activate both F XII and tissue factor to initially form microthrombi and emboli throughout the microvasculature Fibrinolytic disorder Disorder of the fibrinolytic system can lead to hemorrghage when clot breakdown is enhance or excessive .

Management Management • • • • Transfusions Pharmacy Splenectomy Bone marrow transplantation .

Identification of Dental Patient with Bleeding Disorder • History of bleeding problems following surgical procedures • Identification of mediacations with hemostatic effect • Medical conditions • History of heavy alcohol intake .

Laboratory Test .

Oral Findings • • • • Petechie or echymoses Spontaneous gingival bleeding Teeth discoloration (Brown) TMJ problem .

.Dental Management The goal of management is to preoperatively restore the hemostatic system to an acceptable range while supporting coagulation with adjunctive and/or local measures.

Dental Management Dental Management .