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1 Coagulation : Clinical issues Dr. M.B. Agarwal, MD, MNAMS Head, Dept of Haematology, Bombay Hospital Inst of Med Sc, Mumbai 4 th Basic Haemato-Pathology.

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Presentation on theme: "1 Coagulation : Clinical issues Dr. M.B. Agarwal, MD, MNAMS Head, Dept of Haematology, Bombay Hospital Inst of Med Sc, Mumbai 4 th Basic Haemato-Pathology."— Presentation transcript:

1 1 Coagulation : Clinical issues Dr. M.B. Agarwal, MD, MNAMS Head, Dept of Haematology, Bombay Hospital Inst of Med Sc, Mumbai 4 th Basic Haemato-Pathology Course, TMH, Mumbai Friday, 30 th May 2014

2 2 1. Genetic bleeding disorders

3 3 Prakash, 15-year old boy from Pune with post-traumatic nasal bleeding No family h/o bleeding disorder Prakash Kumar : A case study

4 4 Prakash Kumar: Lab investigations TestsResultsControl Hb13.3 g/dl Haematocrit41.4 % WBC7900/cmm Platelets368,000/cmm PT11 s PTT46 s28 s

5 5 Differential diagnosis

6 6 Factor deficiency or Inhibitors

7 7 TestResultControl PTT46 s28 s PTT mix (4:1)30 s

8 8 Factor deficiency

9 9 Normal coagulation cascade

10 10 Intrinsic pathway defect Factor VIII deficiency including Von Willebrand Disease Factor IX deficiency Factor XI deficiency (rare) Factor XII deficiency (non-bleeder)

11 11 TestResultNormal Factor VIII : C9 % % Factor IX77 % % What does this mean ?

12 12 Classical haemophilia A or Von Willebrand Disease

13 13 VWD has equal prevalence in both sexes (Autosomal)

14 14 Prakash Kumar : A case study Bleeding from nose Raised PTT Good correction on mixing studies Low factor VIII : C

15 15 Prakash Kumar : Further work up TestResultNormal VIII : C9%50-150% VWF : Ag12 % % VWF : RCoF10 % %

16 16 Prakash Kumar : Final diagnosis

17 17 Von Willebrand Disease

18 18 VWD : Primary classification SubtypesVWF Type 1Partial deficiency (AD) Type 2Qualitative defect (AD) Type 3Total deficiency (AR)

19 19 VWD type 2 : sub classification SubtypesCharacteristicsDD 2A* High mol wt VWF multimers absent VWD : Type 1 & Type 2M 2B* Low and High mol wt VWF ↓ ↑ RIPA Thrombocytopenia 2M* High mol wt VWF multimers normal ↓ VWF : CB VWD : Type 2A 2N**Markedly ↓ affinity for factor VIIIHaemophilia *VWF : Ag > VWF : RcoF, **AR

20 20 Prakash Kumar : Final diagnosis

21 21 Von Willebrand Disease – Type 1

22 22 2. Acquired bleeding disorders

23 23 Case study Mr. Rajanikant, 69 y from Mumbai SC hematomas & easy bruising : 2 mths Platelet count : 3,32,000/cmm PT : 43/12 secs, INR 3.7 PTT : 56/30 secs Both PT & PTT are raised

24 24 How do you approach a case with both raised PT & PTT ?

25 25 First, we must exclude difficult collection (partially clotted blood)

26 26 We must also exclude effect of high haematocrit (polycythemia)

27 27 Normal coagulation cascade

28 28 We should now consider a single genetic factor deficiency from common pathway or multiple factor deficiencies from both pathways

29 29 Common pathway defect Vitamin K deficiency Chronic liver disease Consumptive coagulopathy Anticoagulant therapy Mr. Rajanikant

30 30 Genetic disorder : Unlikely Vitamin K therapy : No effect Liver function : normal DIC profile : normal Mr. Rajanikant

31 31 Rajanikant : Mixing studies TestPTPTT Raj4356 Control : 1 mix1534 Conclusion : Factor deficiency

32 32 Common pathway defect Factor I deficiency Factor II deficiency Factor V deficiency Factor X deficiency

33 33 Factor I : 290 mg/dl Factor II : 87% Factor V : 78% Factor X : 1.2% Rajanikant : Factor assays

34 34

35 35 Amyloidosis

36 36 Rajanikant : 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

37 37 3. Pictorial Quiz

38 38

39 39 What is this ?

40 40 What is this ?

41 41

42 42

43 43

44 44

45 45

46 46

47 47

48 48 What is it?

49 49

50 50

51 51

52 52

53 53 Blue toe syndrome

54 54

55 55 CoaguloChek

56 56

57 57 Bleeding at 6 pm on left forearm

58 58 Factitious purpura

59 59 Thank You


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