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Hematology And Oncology > Cogulation Disorders > Flashcards

Cogulation Disorders Flashcards

1
Q

PTT

A

Test function of common and intrinsic pathway (all factors except 7 and 13)

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2
Q

Platelets abnormalities - sign symptoms labs

A
  1. Microhemorrhage (mucus bleeding , epistaxis, petechiae, purpura
  2. Increased bleeding time
  3. Possible decreased platelet count
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3
Q

Bernard-Soulier - PC, BT

A

PC: normal or decreased
BT: increased

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4
Q

Bernard-Soulier diagnosis

A

No agglutination on ristocetin cofactor assay

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5
Q

Bernard-Soulier pathophysiology

A

Deficiency of Gp1b (platelets - vWf adhesion)

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6
Q

Bernard-Soulier findings

A
  1. Defects in platelet plug formation

2. Large platelets (younger)

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7
Q

PT

A

Test function of common and extrinsic pathway (factors 1, 2, 5, 7, 10)

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8
Q

Glanzmann thrombasthenia - PC,BT

A

PC: -
BT: increased

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9
Q

Glanzmann thrombasthenia pathophysiology

A

GpIIb/IIIa deficiency –> defects in platelet to platelet aggregation

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10
Q

Glanzmann thrombasthenia labs

A

Blood smear shows no platelet climbing

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11
Q

Glanzmann thrombasthenia - agglutination

A

Agglutation with ristocetin

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12
Q

Immune thrombocytopenia - PC, BT

A

PC: decreased
BT: increased

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13
Q

Immune thrombocytopenia treatment

A
  1. Steroids
  2. IV immunoglobulins
  3. splenectomy (for refractory)
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14
Q

Immune thrombocytopenia labs

A

Increased megakaryocytes on bone bone marrow biopsy

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15
Q

Immune thrombocytopenia pathophysiology

A

Anti GpIIb/IIIa antibodies –> splenic macrophages consumption of platelet antibody complex
Commonly after viral illness

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16
Q

Immune thrombocytopenia is commonly due to

A

Viral illness

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17
Q

Thrombotic thrombocytopenic purpura - PC, BT

A

PC: decreased
BT: increased

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18
Q

Thrombotic thrombocytopenic purpura treatment

A
  1. Plasmapheris

2. Steroids

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19
Q

Thrombotic thrombocytopenic purpura labs and symptoms

A

LDH increased
Schistocytes
symptoms: pentad of neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

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20
Q

Thrombotic thrombocytopenic purpura pathophysiology

A

Inhibition or deficiency of ADAMTS 13 (vwf metalloprotease) –> decreased degradation of vwf multimers –> large vwf multimers –> platelets aggregation and thrombosis

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21
Q

Von willebrand disease - PC, BT, PT, PTT

A

PC normal
BT increased
PT normal
PTT increased or normal

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22
Q

Von willebrand disease mode of inheritance

A

AD

23
Q

MC inherited bleeding disorder

A

Von willebrand disease (but mild)

24
Q

Von willebrand disease diagnosis

A

No agglutination with ristocetin

25
Q

Von willebrand disease - treatment

A

Desmopressin (release vWF stored in endothelium)

26
Q

DIC - PC, BT, PT, PTT

A

PC decreased
BT increased
PT increased
PTT increased

27
Q

Causes of DIC - results in

A

STOP Making New Thrombi
Sepsis (gram -), trauma, obstetric complication, acute pancreatitis, malignancy, nephrotic syndrome, transfusion
results in widespread activation of clootting –> deficiency of factors –> bleeding state

28
Q

DIC labs

A
  1. Schistocytes
  2. Increased d dimers
  3. Decreased fibrinogen
  4. DECREASED FACTORS V AND VIII
29
Q

Prothrombin gene mutation

A

Mutation in 3 untranslated region –> increased production of prothrombin –> increased plasma levels and VENOUS thrombosis

30
Q

Factor V Leiden - definition and mechanism

A
  • Production of mutant factor V, resistant to pr C

- G –> A DNA mutation –> Arg506Gln mutation near the clevage site (from arginine to glutamine)

31
Q

MCC of inherited hypercoagubility in Caucasians

A

Factor V Leiden

32
Q

Protein C or S deficiency pathophysiology

A

Decreased ability to inactivate Va and VIIIa

33
Q

Protein C or S deficiency increases the risk of

A

Thrombotic skin and subcutaneous necrosis with hemorrhage following administration of warfarin

34
Q

Causes of Antithrombin deficiency

A
  1. Inherited deficiency

2. Renal failure/nephrotic syndrome –> loss in urine

35
Q

Antithrombin deficiency pathophysiology

A

Decreased inhibition of factors 2a and 10a

36
Q

Antithrombin deficiency - PTT, PT, Thrombin time

A

no direct effects on the PTT, PT, or thrombin time –> diminishes the increase in PTT following heparin

37
Q

Iron poisoning treatment

A

Chelation (iv deferoxamine, oral deferasixor) and dialysis

38
Q

Iron poisoning mechanism

A

Cell death due to peroxidation of membrane lipids

39
Q

Iron poisoning symptoms and signs

A
  1. Nausea
  2. Vomiting
  3. Gastric bleeding
  4. Lethargy
  5. Scarring leading to GI obstruction
40
Q

Iron poisoning - children

A

High mortality rate with accidental ingestion (adult iron tablets look like candy)

41
Q

INR - definition/MC test in

A

international normalized ratio –> calculated from PT
1=normal
more than 1 = prolonged
MC test used to follow patients on warfarin

42
Q

coagulation disorders - types

A
  1. Hemophilia A, B, C

2. Vitamin K deficiency

43
Q

Vitamin K deficiency - PT, PTT, BT

A

increased PT, PTT

normal BT

44
Q

Vitamin K deficiency –> coagulation disorder due to

A

low activity of factors 2, 7, 9, 10, C, S

45
Q

Hemophilia A, B, C - mechanism

A

Intrinsic pathway coagulation defect
A: deficiency of 8 (XR)
B: deficiency of 9 (XR)
C: deficiency of 11 (AR)

46
Q

Hemophilia A, B, C - PTT, PT

A

normal PT

increased PTT

47
Q

Hemophilia A, B, C - treatment

A

A: desmopressin + factor 8 concentrate
B: factor 9 concentrate
C: Factor 11 concentrate

48
Q

Hemophilia A, B, C - presentation

A

Macrohemorrhage –>hemarthroses (bleeding into joints such as knee), easy bruising, bleeding after trauma or surgery (eg. dental)

49
Q

Hemolytic-uremic syndrome - PC, BT

A

decreased PC

increased BT

50
Q

Hemolytic-uremic syndrome - characterised by

A
  1. thrombocytopenia
  2. microangiopathic hemolytic anemia
  3. acute renal failure
51
Q

Hemolytic-uremic syndrome - typically seen in/due to

A

children, accompanied by diarrhea and commonly caused by Shiga-toxin-producing E-coli (eg. 157:H7)
in adults does not present with diarrhea
STEC infection is not required

52
Q

Hemolytic-uremic syndrome - treatment

A

plasmapheresis

53
Q

Hemolytic-uremic syndrome - symptoms

A

neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

54
Q

Factor V Leiden - complications

A
  1. DVT
  2. cerebral vein thromboses
  3. recurrent pregnancy loss

Hematology And Oncology (21 decks)

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