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Hematology & Oncology > Coagulation & Platelet Disorders > Flashcards

Coagulation & Platelet Disorders Flashcards

1
Q

What does PT test?

A

Function of common & extrinsic pathway (factors I, II, V, VII, X). Inc in a defect

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2
Q

What does PTT test?

A

Function of common & intrinstic pathway (all factors except VII & XIII). Inc in defect

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3
Q

What is the MC coagulation disorder?

A

Hemophilia A

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4
Q

What is increased in Hemophilia A or B?

A

PT

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5
Q

What is the defect in Hemophilia A & B?

A

Intrinsic pathway coagulation defect

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6
Q

What is deficient in Hemophilia A?

A

Factor VIII

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7
Q

What is deficient in Hemophilia B?

A

Factor IX

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8
Q

What are the sx of hemophilia A & B?

A
  • Macrohemorrhages
  • Hemarthroses (bleeding into joints)
  • Easy bruising
  • Inc PTT
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9
Q

What is increased in Vitamin K?

A

PT & PTT

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10
Q

What is the defect in Vitamin K def?

A

General coagulation defect

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11
Q

Which factor synthesis is dec in Vitamin K def?

A

Factors II, VII, IX, X, protein C & S

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12
Q

What do defects in platelet plug formation lead to?

A

Inc bleeding time (BT)

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13
Q

What are the signs/sx of Platelet ABN?

A
  • Microhemorrhage
  • Mucous mem bleeding
  • Epixtaxis
  • Petechiae
  • Purpura
  • Inc bleeding time
  • Possible dec platelet count (PC)
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14
Q

What are the lab features of Bernard-Soulier synd?

A
  • Dec platelet count
  • Inc bleeding time
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15
Q

What is the defect in Bernard-Soulier synd?

A

Platelet plug formation

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16
Q

What is decreased in Bernard-Soulier synd?

A

Dec GpIb→ defect in platelet-to-vWF adhesion

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17
Q

What are the lab features of Glanzmann’s thrombasthenia?

A
  • Inc bleeding time
  • Blood smear shows no platelet clumping
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18
Q

What is the defect in Glanzmann’s thrombasthenia?

A

Defect in platelet plug formation

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19
Q

What is decreased in Glanzmann’s thrombasthenia?

A

GpIIb/IIIa→ platelet-to-platelet aggregation

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20
Q

What are the lab features in Idiopathic thrombocytopenic purpura (ITP)?

A
  • Dec platelet count
  • Inc bleeding time
  • Inc megakaryocytes
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21
Q

What is the defect in ITP?

A

Anti-GpIIb/IIIa Ab’s→ splenic macrophage, consumption of platelet/Ab complex

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22
Q

What is dec in ITP?

A

Platelet survival

23
Q

What are the lab features of Thrombotic thombocytopenic purpura (TTP)?

A
  • Dec platelet count
  • Inc bleeding time
  • Schistocytes
  • Inc LDH
24
Q

What is deficient in TTP?

A

ADAMTS 13 (vWF metalloprotease)→ dec degradation of vWF multimers

25
Q

What is the pathogenesis of TTP?

A

Inc large vWF multimers→ inc platelet aggregation & thrombosis

26
Q

What is dec in TTP?

A

Platelet survival

27
Q

What are the sx of TTP?

A
  • Neurologic sx
  • Renal sx
  • Fever
  • Thrombocytopenia
  • Microangiopathic hemolytic anemia
28
Q

What are the lab features in von Willebrand’s dz?

A
  • Inc bleeding time
  • Normal or inc PTT
29
Q

What is the intrinsic pathway coag defect in Von Willebrand dz?

A

Dec vWF→ normal or inc PTT (depends on severity; vWF acts to carry/protect factor VIII)

30
Q

What is the defect in platelet plug formation in von Willebrand’s dz?

A

Dec vWF→ defect in platelet-to-vWF adhesion

31
Q

What is the MC inherited bleeding disorder?

A

von Willebrand’s dz

(AD)

32
Q

How is von Willebrand’s dz dx?

A

Ristocetin cofactor assay

33
Q

What is the tx of von Willebrand’s dz?

A

DDAVP (desmopressin) which releases vWF stored in endothelium

34
Q

What are the test features in DIC?

A
  • Dec platelet count
  • Inc bleeding time
  • Inc PT
  • Inc PTT
35
Q

What is DIC?

A

Widespread activation of clotting leads to a fef in clotting factors, which creates a bleeding state

36
Q

What are the causes of DIC?

A
  • Sepsis (gram-neg)
  • Trauma
  • Obstetric complications
  • Acute pancreatitis
  • Malignancy
  • Nephrotic synd
37
Q

What are the lab features in DIC?

A
  • Schistocytes
  • Inc fibrin split products (D-dimers)
  • Dec fibrinogen
  • Dec factors V & VIII
38
Q

What is Factor V Leiden dz?

A

Production of mutant factor V that is resistant to degradation by activated protein C

39
Q

What is the MCC of inherited hypercoagulability in whites?

A

Factor V Leiden dz

40
Q

What is Prothrombin gene mutation?

A

Mutation in 3’ untranslated region→ inc production of prothrombin→ inc plasma levels & venous clots

41
Q

What is Antihrombind def?

A

Inherited def of antithrombin

42
Q

What is blunted after heparin administration in Antithrombin def?

A

Inc in PTT

43
Q

What is Protein C or S def?

A

Dec ability to inactivate factors V & VIII

44
Q

What does Protein C or S def inc risk of?

A

Thrombotic skin necrosis w/ hemorrhage following administration of warfarin

45
Q

What is the dosage effect of Packed RBCs?

A

Inc Hb & O2 carrying capacity

46
Q

What is the clinical use of Packed RBC’s?

A
  • Acute blood loss
  • Severe anemia
47
Q

What is the dosage effect of Platelets?

A

Inc platelet count (Inc -5,000/mm3/unit)

48
Q

What is the clinical use of Platelets?

A

Stop significant bleeding (thrombocytopenia, qualitative platelet defects)

49
Q

What is the dosage effect of Fresh frozen plasma?

A

Inc coagulation factor levels

50
Q

What are the clinical uses for Fresh frozen plasma?

A
  • DIC
  • Cirrhosis
  • Warfarin overdose
51
Q

What is the dosage effect of Cryoprecipitate?

A

Contains fibrinogen, factor VIII, factor XIII, vWF & fibronectin

52
Q

What are the clinical uses of Cyroprecipitate?

A

Tx coagulation factor def involving fibrinogen & factor VIII

53
Q

What are the risks of blood transfusion?

A
  • Include infection transmission (low)
  • Transfusion reactions
  • Iron overload
  • Hypocalcemia (citrate is a Ca chelator)
  • Hyperkalemia (RBC may lyse in old blood unit)

Hematology & Oncology (7 decks)

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