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FA - Hematology and Oncology > Hem & Onc - Pathology (Coagulation & platelet disorders, Hereditary hypercoagulability, & Blood transfusion therapy) > Flashcards

Hem & Onc - Pathology (Coagulation & platelet disorders, Hereditary hypercoagulability, & Blood transfusion therapy) Flashcards

Pg. 389-391 in First Aid 2014 or Pg. 359-360 in First Aid 2013 Sections include: -Coagulation disorders -Platelet disorders -Mixed platelet and coagulation disorders -Hereditary thrombosis syndromes leading to hypercoagulability -Blood transfusion therapy

1
Q

Which coagulation pathways and factors does PT test?

A

Tests function of common and extrinsic pathway (i.e., factors I, II, V, VII, and X)

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2
Q

Which coagulation pathways and factors does PTT test?

A

Tests function of common and intrinsic pathway (all factors except VII and XIII)

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3
Q

In general, what kind of defect causes Hemophilia A or B? What are the specific defects?

A

Both intrinsic pathway coagulation defects; A: deficiency of factor VIII –> increased PTT; B: deficiency of factor IX –> increased PTT

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4
Q

What are the PT and PTT trends in Hemophilia A or B?

A

PT normal, PTT increased

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5
Q

What are the symptoms associated with Hemophilia A or B?

A

Macrohemorrhage in hemphilia - hemarthroses (bleeding into joints), easy bruising

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6
Q

What is the lab finding associated with Hemophilia A or B?

A

increased PTT

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7
Q

What is the treatment for hemophilia A?

A

Treatment: Recombinant factor VIII (in Hemophilia A)

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8
Q

What are PT and PTT trends in Vitamin K deficiency? What is the bleeding time associated with Vitamin K deficiency?

A

Both PT and PTT increase; Normal bleeding time

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9
Q

What kind of defect does Vitamin K deficiency cause?

A

General coagulation defect

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10
Q

Vitamin K deficiency decreases the synthesis of what substances?

A

Decrease synthesis of factors II, VII, IX, X, protein C, protein S

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11
Q

What causes an increased bleeding time?

A

Defects in platelet plug formation –> increased bleeding time (BT)

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12
Q

Name 3 effects of platelet abnormalities.

A

Platelet abnormalities –> (1) microhemorrhage: mucus membrane bleeding, epistaxis, petechiae, purpura, (2) increased bleeding time, (3) possible decreased platelet count (PC)

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13
Q

Give 4 examples of microhemorrhage that may occur as a result of platelet abnormalities.

A

microhemorrhage: mucus membrane bleeding, epistaxis, petechiae, purpura

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14
Q

What changes (if any) occur in platelet count (PC) and bleeding time (BT) with Bernard-Soulier syndrome?

A

PC decreased, BT increased

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15
Q

In general, what kind of defect is Bernard-Soulier syndrome? What is the specific defect, and what causes it?

A

Defect in platelet plug formation; Decreased GpIb –> defect in platelet-to-vWF adhesion

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16
Q

In general, what kind of defect is Glanzmann thrombasthenia? What is the specific defect, and what causes it?

A

Defect in platelet plug formation; Decreased GpIIb/IIIa –> defect in platelet-to-platelet aggregation

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17
Q

What lab finding is associated with Glanzmann thrombasthenia?

A

Labs: blood smear shows no platelet clumping

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18
Q

What changes (if any) occur in platelet count (PC) and bleeding time (BT) with Glanzmann thrombasthenia?

A

PC = no change, BT increased

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19
Q

What changes (if any) occur in platelet count (PC) and bleeding time (BT) with Immune thrombocytopenia?

A

PC decreased, BT increased

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20
Q

What is the specific defect in Immune thrombocytopenia, and what effect does it have?

A

Defect: anti-GpIIb/IIIa antibodies –> splenic macrophage consumption of platelet/antibody complex.

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21
Q

What may trigger Immune thrombocytopenia?

A

May be triggered viral illness.

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22
Q

How are platelets affected in Immune thrombocytopenia?

A

Decreased platelet survival

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23
Q

What lab finding is associated with Immune thrombocytopenia?

A

Labs: Increased megakaryocytes on bone marrow biopsy

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24
Q

What changes (if any) occur in platelet count (PC) and bleeding time (BT) with Thrombocytic thrombocytopenic purpura?

A

PC decreased, BT increased

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25
Q

What is the specific defect in Thrombocytic thrombocytopenic purpura, and what effect does it have?

A

Inhibition or deficiency of ADAMTS 13 (vWF metalloprotease) –> decreased degradation of vWF multimers

26
Q

What is the pathogenesis of Thrombocytic thrombocytopenic purpura?

A

Pathogenesis: increased large vWF multimers –> increased platelet adhesion –> increased platelet aggregation and thrombosis

27
Q

How are platelets affected in Thrombocytic thrombocytopenic purpura?

A

Decreased platelet survival

28
Q

What are 2 lab findings associated with Thrombocytic thrombocytopenic purpura?

A

Labs: schistocytes, increased LDH

29
Q

What are the symptoms associated with Thrombocytic thrombocytopenic purpura?

A

Symptoms: pentad of neurologic and renal symptoms, fever, thrombocytopenia, and microangiopathic hemolytic anemia

30
Q

What is the treatment for Thrombocytic thrombocytopenic purpura?

A

Treatment: exchange transfusion and steroids

31
Q

What are the changes to the following values in von Willebrand disease: (1) PC (2) BT (3) PT (4) PTT?

A

(1) No change (2) Increased (3) No change (4) No change or Increased

32
Q

What are the changes to the following values in DIC: (1) PC (2) BT (3) PT (4) PTT?

A

(1) Decreased (2) Increased (3) Increased (4) Increased

33
Q

What are the defects associated with von Willebrand disease, and what effects do they have?

A

(1) Intrinsic pathway coagulation defect: decreased vWF –> normal or increased PTT (depends on severity; vWF acts to carry/protect factor VIII). (2) Defect in platelet plug formation: decreased vWF –> defect in platelet-to-vWF adhesion.

34
Q

How severe is von Willebrand disease, and how common is it?

A

Mild but most common inherited bleeding disorder.

35
Q

What is the mode of inheritance of von Willebrand disease?

A

Autosomal dominant

36
Q

How is von Willebrand disease diagnosed?

A

Diagnosed in most cases by ristocetin cofactor assay (decreased agglutination is diagnostic)

37
Q

What is the treatment for von Willebrand disease, and how does it work?

A

Treatment: DDAVP, which releases vWF stored in endothelium

38
Q

What is the pathogenesis of DIC?

A

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

39
Q

What are 7 causes of DIC?

A

Causes: (1) Sepsis (gram-negative) (2) Trauma (3) Obstetric complications (4) acute Pancreatitis (5) Malignancy (6) Nephrotic syndrome (7) Transfusion; Think: “STOP Making New Thrombi”

40
Q

What are 5 lab findings associated with DIC?

A

Labs: (1) schistocytes (2) increased fibrin split products (D-dimers) (3) decreased fibrinogen (4) decreased factors V and (5) VIII

41
Q

What is the most common cause of inherited hypercoagulability in whites?

A

Factor V Leiden

42
Q

What causes Factor V Leiden, and what is its effect?

A

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

43
Q

Where is the Prothrombin gene mutation, and what effects does it have?

A

Mutation in 3’ untranslated region –> increased production of prothrombin –> increased plasma levels and venous clots

44
Q

What effects does inherited Antithrombin deficiency have versus not have?

A

Inherited deficiency of antithrombin: has no direct effect on the PT, PTT, or thrombin time but diminishes the increase in PTT following heparin administration.

45
Q

What are 4 hereditary thrombosis syndromes leading to hypercoagulability?

A

(1) Factor V Leiden (2) Prothrombin gene mutation (3) Antithrombin deficiency (4) Protein C or S deficiency

46
Q

What is the cause and effect of acquired Antithrombin deficiency?

A

Can also be acquired: renal failure/nephrotic syndrome –> antithrombin loss in urine –> increased factors II and X

47
Q

What effect does Protein C or S deficiency have?

A

Decreased ability to activate factors V and VIII

48
Q

For what outcome does Protein C or S deficiency increase the risk, and in what context?

A

Increased risk of thrombotic skin necrosis with hemorrhage following administration of warfarin

49
Q

If a patient has skin and subcutaneous tissue necrosis after Warfarin administration, what condition should be on the differential diagnosis?

A

Skin and subcutaneous tissue necrosis after warfarin administration –> think protein C deficiency.; Think: “Protein C Cancels Coagulation”

50
Q

What are 4 types of blood transfusion therapy?

A

(1) Packed RBCs (2) Platelets (3) Fresh frozen plasma (4) Cryoprecipitate

51
Q

What is the dosage effect of blood transfusion therapy containing packed RBCs?

A

Increase Hb and O2 carrying capacity

52
Q

What is the dosage effect of blood transfusion therapy containing platelets?

A

Increase platelet count (increase ~5000/mm^3/unit)

53
Q

What is the dosage effect of blood transfusion therapy containing fresh frozen plasma?

A

Increase coagulation factor levels

54
Q

What is the dosage effect of blood transfusion therapy containing cryoprecipitate?

A

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

55
Q

What are 2 clinical uses of blood transfusion therapy containing packed RBCs?

A

(1) Acute blood loss (2) Severe anemia

56
Q

What is the general clinical use of blood transfusion therapy containing platelets? Give 2 specific examples of this use.

A

Stop significant bleeding (thrombocytopenia, qualitative platelet defects)

57
Q

What are 4 clinical uses of blood transfusion therapy containing fresh frozen plasma?

A

(1) DIC (2) Cirrhosis (3) Warfarin overdose (4) Exchange transfusion in TTP/HUS

58
Q

What is the clinical use of blood transfusion therapy containing cryoprecipitate?

A

Treat coagulation factor deficiencies involving fibrinogen and factor VIII

59
Q

What are 5 risks of blood transfusions?

A

Blood transfusion risks include (1) infection transmission (low) (2) transfusion reactions (3) iron overload (4) hypocalcemia (citrate is a calcium chelator) (5) hyperkalemia (RBCs may lyse in old blood units).

60
Q

Why is hypocalcemia a risk of blood transfusions?

A

Hypocalcemia (citrate is a calcium chelator)

61
Q

Why is hyperkalemia a risk of blood transfusions?

A

Hyperkalemia (RBCs may lyse in old blood units)

FA - Hematology and Oncology (17 decks)

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