Oral manifestations of Hematologic Disorders

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					Oral manifestations of
Hematologic Disorders
    Ajiravudh Subarnbhesaj
       Hematologic disorders
 Disorders of Red Blood Cells
 Disorders of White Blood Cells
 Bleeding Disorders
Red Blood Cells (Erythrocytes)
    Disorders of Red Blood Cells
 Anemia
 Polycythemia
                       Anemia

: A reduction in the oxygen-carrying
 capacity of the blood
 : Usually related to a decrease in number
 of circulating RBCs or to an abnormality in
 the Hb contained within the RBCs
      Male hemoglobin level : 13.5 g/dl
      Female hemoglobin level : 11.0 g/dl
    Classification of Red Blood Cells Disorders

 Iron deficiency anemia
 Folate deficiency anemia
 Glucose-6-Phosphate Dehydrogenase Deficiency
 Sickle cell anemia
 Renal disease
             Iron-deficiency anemia
 Microcytic anemia
 Most common cause of anemia in developed
  countries
 caused by excessive blood loss (menses,
  bleeding from the GI tract, malabsorption
  syndrome)
 Children (poor dietary intake), Women
  (menstrating or pregnant), Men (GI bleeding or
  malignancy)
   Asymptomatic – pallor   (oral mucosa, conjunctiva, nail bed)
              Folate-deficiency anemia &
       Pernicious anemia (Cobalamin deficiency)

 Macrocytic anemia
 Vitamin B12 (Cobalamin) and Folic acid are both
  needed for the maturation of RBCs in bone
  marrow (Why and How?)
 Causes – celiac disease or tropical sprue
 The absorption of folate is interfered by alcohol
  consumption and certain drug (methotrexate)
   Pernicious anemia is a disease of late adult life
    (40-70 yrs)
            G-6-PD deficiency anemia

 Hemolytic anemia
 G-6-PD is an enzyme needed for the hexose
  monophosphate shunt pathway
 Blockage of the hexose monophosphate shunt pathway
  allows the accumulation of oxidants in the RBCs – Heinz
  bodies – hemolysis
 Clinical features – intravascular hemolysis  severe
  jaundice, dyspnea and dizziness
 Drugs which can trigger hemolysis – aspirin, phanacetin,
  dapsone, ascorbic acid and vitamin K
 Fava bean – most comon cause
                  Sickle cell anemia

 Sickle cell hemoglobin is the first variant of Hb to be
  recognised of more than 600 inherited human Hb
  variants (hemoglobinopathies)
 More commonly found in regions of malarial endemicity
 Autosomal recessive trait
 Homozygous state – a gene from each parent
  contributes to formation of the HbS molecule
 Sickle-shaped RBC – deoxygenation or decreased blood
  pH
 Management – penicillin prophylaxis is used for at least
  the first 5 years of life
 Severe case – high doses of folic acid, analgesics,
  hydration and blood transfusions
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                 Renal disease

   The kidney produces the hormone
    erythropoietin, which stimulates RBC
    production by the bone marrow
            Polycythemia
 Increased number of circulating RBCs
 Primary and Secondary (?)
Signs and Symptoms of Red Blood Cells
             Disorders




     Koilonychia      Pale conjunctiva
Oral manifestations of Red Blood Cells
              Disorders




Angular cheilitis     Depapillated tongue
         Screening lab tests
 Complete Blood Count and differential
 Hb level, hematocrit, MCV
 Total WBC count and platelet count
          White Blood Cells
 WBCs found in the peripheral circulation –
  granulocytes, lymphocytes and monocytes
 Granulocytes – 90% => neutrophils
  followed by eosinophils and basophils
 Lymphocytes – T cell, B cell and natural
  killer cells (NK cell)
 The majority of the WBCs are produced
  primarily in the bone marrow
    Classification of White Blood Cells
                 Disorders
 Leukocytosis
 Leukopenia
 Myeloproliferative disorders
 Lymphoproliferative disorders
             Leukocytosis
 Increased number of circulating WBCs
 Pregnancy and emotional stress –
  physiologic leukocytosis
 Infections (parasitic), neoplasia and
  necrosis – pathologic leukocytosis
             Leukopenia
 Decreased number of circulating WBCs
  (less than 4500/mm3 )
 Early phase of leukemia and lymphoma
 Agranulocytosis and pancytopenia due to
  toxic effects of drugs and chemicals
 Cyclic neutropenia (every 21-28 days) –
  infections and oral manifestations
Neutropenia
    Myeloproliferative disorders
 Acute myeloid leukemia
  : immature neoplastic malignancy of myeloid cells
 Chronic myeloid leukemia
  : mature neoplastic malignancy of myeloid cells
    Lymphoproliferative disorders
   Acute lymphoblastic leukemia
    : immature neoplastic malignancy of lymphoid cells

   Chronic lymphocytic leukemia
    : mature neoplastic malignancy of lymphoid cells

   Lymphomas
    Hodgkin’s disease: malignant growth of lymphocytes primarily in LNs
    Non-Hodgkin’s disease: B or T cell malignant neoplasms, many types and
    locations
    Burkitt’s lymphoma: B cell malignancy involving bone and lymph nodes

   Multiple myeloma
    : overproduction of malignant plasma cells involving bone
Acute lymphoblastic leukemia




Acute myelocytic leukemia
Multiple myeloma
: punch-out lesion




Histopathology of
Multiple myeloma
Oral ulceration
        Bleeding Disorders
 Nonthrombocytopenic purpura
 Thrombocytopenic purpura
 Disorders of coagulation
    Nonthrombocytopenic purpura
    Vascular wall      Disorders of platelet
     alteration          function
      Scurvy                   Genetic defects
      Infections               Drugs
      Chemicals          (Aspirin, NSAIDs, Alcohol, Beta-lactam
                         antibiotics, Penicillin, Cephalotins)
      Allergy
                              Allergy
                              Autoimmune disease
                              von Willebrand’s disease
                         (secondary factor VIII disease)
                              Uremia
Clotting Diagram
    Thrombocytopenic purpura
 Primary-idiopathic
 Secondary
     Chemicals
     Physical agents (radiation)
     Systemic disease (leukemia)
     Metastatic cancer
     Splenomegaly
     Drugs
       Alcohol, Thiazide diuretics, Estrogens, Gold salt
     Vasculitis
     Mechanical prosthetic heart valves
     Viral or bacterial infections
          Disorders of coagulation
   Inherited                        Acquired
    Hemophilia A (def fac VIII)       Liver disease
    Hemophilia B (def fac IX)         Vitamin deficiency
    Others                                  Biliary tract obstruction
                                            Malabsorption
                                            Excessive use of broad-
                                      spectrum antibiotics
                                      Anticoagulation drugs
                                            Heparin
                                            Coumarin
                                            Aspirin and NSAIDs
                                      DIC
                                      Primary fibrinogenolysis
   Vascular wall alteration
Scurvy
Infections
Chemicals
Allergy
Syndromes
   Osler-Weber-Rendu
   Ehlers-Danlos syndrome
   OI
   Marfan syndrome
Scurvy
    Disorders of platelet function
 Von Willebrand’s disease
     An inherited defect involving platelet
     adhesion
 Bernard-Soulier Disease
     (the platelets are defective)
Thrombocytopenic purpura




             Thrombocytopenic purpura
ITP
     Disorders of coagulation
 Inherited
 Acquired
               Hemophilia
 A and B
 A (factor VIII) -> X-linked recessive trait
 B (factor IX) -> Christmas disease
                 -> X-linked recessive trait
 Bleed extensively from trivial injuries
 Hemarthrosis, spontaneous bleeding,
  intracarnial hemorrhage
 Soft tissue hematoma
If the father is hemophiliac
and the mother is
normal……..
What will happen to the
children?
Queen Victoria of England   Czar Nicolas 2nd of Russia
Hemarthrosis
Subcutaneous bleeding
after an injection
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posted:5/1/2012
language:English
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