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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

23
Q

MC inherited bleeding disorder

A

Von willebrand disease (but mild)

24
Q

Von willebrand disease diagnosis

A

No agglutination with ristocetin

25
Von willebrand disease - treatment
Desmopressin (release vWF stored in endothelium)
26
DIC - PC, BT, PT, PTT
PC decreased BT increased PT increased PTT increased
27
Causes of DIC - results in
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
DIC labs
1. Schistocytes 2. Increased d dimers 3. Decreased fibrinogen 4. DECREASED FACTORS V AND VIII
29
Prothrombin gene mutation
Mutation in 3 untranslated region --> increased production of prothrombin --> increased plasma levels and VENOUS thrombosis
30
Factor V Leiden - definition and mechanism
- Production of mutant factor V, resistant to pr C | - G --> A DNA mutation --> Arg506Gln mutation near the clevage site (from arginine to glutamine)
31
MCC of inherited hypercoagubility in Caucasians
Factor V Leiden
32
Protein C or S deficiency pathophysiology
Decreased ability to inactivate Va and VIIIa
33
Protein C or S deficiency increases the risk of
Thrombotic skin and subcutaneous necrosis with hemorrhage following administration of warfarin
34
Causes of Antithrombin deficiency
1. Inherited deficiency | 2. Renal failure/nephrotic syndrome --> loss in urine
35
Antithrombin deficiency pathophysiology
Decreased inhibition of factors 2a and 10a
36
Antithrombin deficiency - PTT, PT, Thrombin time
no direct effects on the PTT, PT, or thrombin time --> diminishes the increase in PTT following heparin
37
Iron poisoning treatment
Chelation (iv deferoxamine, oral deferasixor) and dialysis
38
Iron poisoning mechanism
Cell death due to peroxidation of membrane lipids
39
Iron poisoning symptoms and signs
1. Nausea 2. Vomiting 3. Gastric bleeding 4. Lethargy 5. Scarring leading to GI obstruction
40
Iron poisoning - children
High mortality rate with accidental ingestion (adult iron tablets look like candy)
41
INR - definition/MC test in
international normalized ratio --> calculated from PT 1=normal more than 1 = prolonged MC test used to follow patients on warfarin
42
coagulation disorders - types
1. Hemophilia A, B, C | 2. Vitamin K deficiency
43
Vitamin K deficiency - PT, PTT, BT
increased PT, PTT | normal BT
44
Vitamin K deficiency --> coagulation disorder due to
low activity of factors 2, 7, 9, 10, C, S
45
Hemophilia A, B, C - mechanism
Intrinsic pathway coagulation defect A: deficiency of 8 (XR) B: deficiency of 9 (XR) C: deficiency of 11 (AR)
46
Hemophilia A, B, C - PTT, PT
normal PT | increased PTT
47
Hemophilia A, B, C - treatment
A: desmopressin + factor 8 concentrate B: factor 9 concentrate C: Factor 11 concentrate
48
Hemophilia A, B, C - presentation
Macrohemorrhage -->hemarthroses (bleeding into joints such as knee), easy bruising, bleeding after trauma or surgery (eg. dental)
49
Hemolytic-uremic syndrome - PC, BT
decreased PC | increased BT
50
Hemolytic-uremic syndrome - characterised by
1. thrombocytopenia 2. microangiopathic hemolytic anemia 3. acute renal failure
51
Hemolytic-uremic syndrome - typically seen in/due to
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
Hemolytic-uremic syndrome - treatment
plasmapheresis
53
Hemolytic-uremic syndrome - symptoms
neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia
54
Factor V Leiden - complications
1. DVT 2. cerebral vein thromboses 3. recurrent pregnancy loss

Hematology And Oncology (21 decks)

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