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BLEEDING DISORDERS

BLEEDING DISORDERS. Dr. Lai Yin Win Chai Pathologist. Definition A group of disorders having an abnormal tendency to bleed due to a defect in the haemostatic mechanism. Characteristics of Abnormal Bleeding Tendency Spontaneous bleeding into skin/ mucous membrane/ internal organs

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BLEEDING DISORDERS

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  1. BLEEDING DISORDERS Dr. Lai Yin Win Chai Pathologist

  2. Definition • A group of disorders having an abnormal tendency to bleed due to a defect in the haemostatic mechanism. Characteristics of Abnormal Bleeding Tendency • Spontaneous bleeding into skin/ mucous membrane/ internal organs • Excessive or prolonged bleeding following trauma or surgery • Bleeding from more than one site

  3. Classification of Haemorrhagic Disorders • Vascular defect • Platelet defect • Coagulation defect

  4. Platelet Defect

  5. Thrombocytopenia Definition • Reduction of platelets in the peripheral blood below the lower normal limit of 150x109/L • N value – 150 – 400 x 109 /L Classification Primary - Immune Thrombocytopenic Purpura Secondary • Acute leukemia • Drugs and chemicals • Aplastic anaemia • Secondary BM infiltration • Hypersplenism • SLE

  6. ImmuneThrombocytopenicPurpura (ITP) Definition An acquired quantitative platelet defect due to autoimmune destruction of mature platelets in the peripheral blood.

  7. Clinical features • Two types- Acute ITP - Chronic ITP

  8. Clinical features • Features of anaemia corresponds to the severity of bleeding. • No jaundice, no fever, Mild splenomegaly • No hepatomegaly nor lymphadenopathy • No gum hypertrophy nor sternal tenderness

  9. Course of bleeding • Spontaneous bleeding – skin (petechiae and ecchymosis) & mucosal (epistaxis & gum bleeding, haematuria, menorrhagia, melaena) and internal organ (brain &spinal cord) relatively uncommon but give serious consequence • Trauma/minor surgery/dental procedure with prolonged excessive bleeding • Severity of bleeding correlates with the degree of thrombocytopenia • Haemarthrosis and wound bleeding – not a feature of thrombocytopenia.

  10. Skin Bleeding

  11. Mucous membrane bleeding

  12. Laboratory Investigations Screening Tests • Bleeding time is prolonged (Normal – 2-7 minutes) • Hess test positive • Normal clotting time Blood for Complete Picture • Hb level – Decreased • Total WBC count – Increased (during bleeding) Peripheral blood film • RBC – Normochromic normocytic Anaemia • WBC – Neutrophil leucocytosis • Platelets – reduced

  13. Specific tests • Platelet count (Thrombocytopenia - < 150 x 109/L) • Exclude qualitative platelet defect • Does not differentiate ITP from secondary thrombocytopenia Bone marrow aspiration • Excludes secondary Thrombocytopenia • In ITP : Megakaryopoiesis – Increased predominately immature megakaryocytes characterised by less cytoplasmic granularity poor platelet budding and vacuolation • Erythroid hyperplasia • Active granulopoiesis Demonstration of IgG Antiplatelet Auto Antibody • Confirms ITP Treatment .Corticosteroid therapy . High dose intravenous Ig therapy

  14. Peripheral blood picture showing giant platelets

  15. Normal Megakaryocyte Abnormal Megakaryocyte in ITP

  16. Bone marrow picture showing megakaryopoietic hyperplasia

  17. Coagulation Disorders Classification Congenital coagulation disorders (1) Haemophilias Haemophilia A or Classical Haemophilia (Factor VIII deficiency) Haemophilia B or Christmas disease (Factor IX deficiency) (2) Von Willebrand disease (3) Others - Factor I deficiency Factor V, VII, X, XI, XII & XIII deficiencies Acquired coagulation disorders • DIC • Liver disease • Vitamin K deficiency • Acute primary fibrinolysis • Massive transfusion of stored blood • Anti-coagulant therapy • Circulating inhibitors of coagulation

  18. XX XY XY XX XX XY XX XX XY XY XX Haemophilia Definition • A group of congenital coagulation disorders leading to an abnormal bleeding tendency due to genetically determined sex-linked recessive defect. • Haemophilia A ( Factor VIII deficiency ) • Haemophilia B ( Factor IX deficiency ) • Males are affected. Females are healthy carriers. XY

  19. History • A male child with history of abnormal bleeding in early infancy or adolescence. • Bleeding is trauma associated. Spontaneous bleeding less common. • Insidious onset, delayed bleeding, recurrent bleeding. • Family history of abnormal bleeding in male relatives from the maternal side of the family.

  20. Clinical Features Due to abnormal bleeding • Types & Sites of bleeding • Wound bleeding - Haematoma formation within deep tissues presenting as a painful swelling. • Joint bleeding – recurrent haemarthrosis is a characteristic feature (the joints usually involved are knee joints & ankle joints leading to pain and disability) • Post - traumatic bleeding & post - surgical bleeding following minor surgery & tooth extraction – prolonged & excessive bleeding. • Skin bleeding (petechial & ecchymosis) are rare and mucosa bleeding can be present. Features of anemia depend on the severity of bleeding.

  21. Joint deformity

  22. Lateral View Front View Haematoma Haematoma

  23. Large Haematoma

  24. Laboratory Investigations Screening Tests • Clotting time prolonged (Normal – 5 – 11minutes) • Bleeding time normal • Hess test normal Blood for complete picture • Hb level - reduced Peripheral blood film • Normochromic normocytic anemia • Neutrophil leucocytosis • Platelets – adequate

  25. Special Tests (1) Prothrombin Time (OSPT) - normal (excludes extrinsic pathway of coagulation - II, V, VII, X) (2) APTT (Activated Partial Thromboplastin Time) – prolonged This indicates intrinsic pathway of coaguation (XII, XI, VIII, IX, X) are defective, most likely Factor VIII or IX (3) Thrombin Time - normal Confirmatory Tests To demonstrate the specific deficient factor as well as to determine the degree of deficiency. • TGT (Thromboplastin Generation Test) to distinguish between Factor VIII & IX • Specific factor assay Treatment – Factor replacement therapy

  26. Disseminated Intravascular Coagulation, DIC(Consumption coagulapathy / Defibrination Syndrome) Definition • Acquired thrombo-haemorrhagic disorder due to secondary complications with a wide variety of clinical settings. • Thrombotic element in DIC is widespread disseminated microvascular thrombosis • Initiated by the following clinical conditions 1. Obstretrical Complications - 50% • Abruptio Placentae (APH) • Amniotic fluid embolism • Septic Abortion • Toxaemia of Pregnancy • Retained Dead Foetus

  27. 2. Infections • Gram (-) Septicaemic Shock, Meningococcaemia, Malaria (Falciparum), Angioinvasive Fungal Infection 3. Neoplastic Conditions • Advanced Mucin producing Adenocarcinoma • Carcinoma Pancreas/Prostate/Lung/stomach • Acute promyelocytic leukaemia (M3) 4. Massive Tissue Injuries • Massive Trauma, Crush Injury, Extensive Burns & Heart & Lung Surgery 5. Miscellaneous • Haemolytic Transfusion Reaction, Acute Intravascular Haemolysis, Snake Bite (Viper Bite)

  28. Clinical Features • Widespread microthrombosis leads to pain (microinfarction) & microangiopathic haemolytic anaemia • Haemorrhagic diathesis follows as localised bleeding from venepuncture sites, operative site & birth canal • Generalised bleeding from skin & mucous membrane

  29. Investigations Blood for complete picture • Hb level – Reduced Peripheral Blood Film • Anaemia – normochromatic normocytic anaemia, fragmented RBCs (MicroAngiopathic Haemolytic Anaemia) • Neutrophil Leucocytosis (Active Bleeding) • Platelets - reduced Screening Tests • Bleeding time – prolonged • Hess test – positive • Clotting time – prolonged • Prothrombin time – prolonged (Lack of Factors II, V, X) • Activated Partial Thromboplastin Time – prolonged (Lack of Factors V, VIII, X) • Thrombin time – prolonged (Lack of Factor I) • Fibrin Degradative Product – increased

  30. Treatment • Treat the underlying cause is the important factor • Bleeding – give platelet concentrate and fibrinogen • Thrombosis – heparin & antiplatelet drugs

  31. Vitamin K Deficiency • It is a fat soluble vitamin which is essential for the synthesis by the liver of factor II, VII, IX, X. • Vitamin K deficiency may present in the newborn or in later life. • In new born, vitamin K level is low due to liver cell immaturity & lack of gut bacterial synthesis of vitamin. • Bleeding manifestations in adults due to obstructive jaundice, pancreatic disease or small bowel disease

  32. Investigations • Bleeding time & Hess test – normal • Clotting time – prolonged • Prothrombin time – prolonged • Activated Partial Thromboplastin Time – prolonged Treatment Intravenous Vitamin K adminstration

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