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Coagulation : Case based approach

Coagulation : Case based approach. 2 nd Basic hematopathology course, TMH, Mumbai Saturday, 11 th June 2011. Dr. M.B. Agarwal , MD, MNAMS Head, Dept of Haematology, Bombay Hospital Inst of Med Sc, Mumbai. Bleeding disorders. Case 1.

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Coagulation : Case based approach

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  1. Coagulation : Case based approach 2nd Basic hematopathology course, TMH, Mumbai Saturday, 11th June 2011 Dr. M.B. Agarwal, MD, MNAMS Head, Dept of Haematology, Bombay Hospital Inst of Med Sc, Mumbai

  2. Bleeding disorders

  3. Case 1

  4. Jigar, 15-year old boy from Vadodara with post-traumatic nasal & oral bleeding : 2 hours • No family h/o bleeding disorder

  5. Jigar : Lab. investigations

  6. Differential diagnosis

  7. Factor deficiencyorInhibitors

  8. What does this mean ?

  9. Factor deficiency

  10. What does this mean ? (46 + 28 = 74 ÷ 2 = 37)

  11. Inhibitors Factor specific Lupus type

  12. Inhibitors Factor specific Lupus type • KCT • dRVVT • L1 / L2 > 1.3 • Factor assay

  13. Normal coagulation cascade

  14. Intrinsic pathway defect • Factor VIII deficiency including von Willebrand Disease • Factor IX deficiency • Factor XI deficiency (rare) • Factor XII deficiency (non-bleeder)

  15. What does this mean ?

  16. Classical haemophilia Aorvon Willebrand Disease

  17. vWD has equal prevalence in both sexes (Autosomal)

  18. Coming back to Jigar • Bleeding from nose • Raised PTT • Good correction on mixing studies • Low factor VIII : C

  19. Jigar : Further work up

  20. Jigar : Final diagnosis

  21. Von Willebrand Disease

  22. vWD : Primary classification

  23. vWD type 2 : sub classification *vWF : Ag > vWF : Rco, **AR

  24. vWD : Laboratory tests • CBC, platelet count, PT : Normal • PTT : often normal • Factor VIII (coagulometer) • vWF : RCoF (aggregometry) • vWF : CB (ELISA) • vWF : Ag (ELISA) • RIPA : Aggregometry • vWF : Multimer analysis (gel electrophoresis)

  25. Variations in vWF levels () • Exercise and stress (adrenaline) • Infection and inflammation • Liver disease • Pregnancy • Estrogen and progesterone therapy • Hyperthyroidism

  26. Genetic variations in vWF levels • Blood group O has lowest level • Black race has higher level • Black with non A may have double the level of a Caucasian with group O • Degree of proteolysis varies • ADAMTS-13 activity varies

  27. Platelet-type vWD • It is not vWD • A genetic platelet disorder (AD) • A gain-of-function mutation •  affinity of platelet GP1b for vWF •  RIPA • Thrombocytopenia • Phenotype : similar to type 2B • Treatment : platelet transfusion and not vWF

  28. Acquired vWD • Not an uncommon disease • Pts with autoimmune disorders, hypothyroidism, lymphoma, WM • Antibodies are present only in a minority • Excessive proteolysis or consumption (AS)

  29. Associations • Angiodysplastic lesions in gut •  Atherosclerosis (pigs) • Diagnosis may be totally missed during pregnancy and early puerperium • Factor VIII level may vary within the family • Role of PFA-100

  30. Case 2

  31. Case study • Mr. Raj, 69y from Rajkot • SC hematomas & easy bruising : 2 mths • Platelet count : 3,32,000/cmm • PT : 43/12 secs, INR 3.7 • PTT : 56/30 secs

  32. What is the differential diagnosis ?

  33. Whenever PT & PTT both are raised ……?

  34. First, we must exclude difficult collection (partially clotted blood)

  35. We must also excludeeffect of high haematocrit (polycythemia)

  36. Common pathway defect • Vitamin K deficiency • Chronic liver disease • Consumptive coagulopathy • Anticoagulant therapy

  37. Liver function : normal • DIC profile : normal

  38. Raj : Mixing studies

  39. Factor I : 290 mg/dl Factor II : 87% Factor V : 78% Factor X : 1.2%

  40. What is the aetiology of selective factor X deficiency at the age of 69 years ?

  41. Amyloidosis

  42. Raj : Amyloidosis • Macroglossia • S. Protein electrophoresis : Faint M band present • S. Immunofixation : Lambda monoclonal gammopathy • Bone marrow : Plasma cells : 2-3% • Bone marrow biopsy : Amyloidosis • Abdominal fat pad biopsy : Amyloidosis

  43. Part 3 Pictorial quiz

  44. What is this ?

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