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Hemostasis > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What factor is deficient in Hemophilia A?

A

factor VIII

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2
Q

What factor is deficient in Hemophilia B?

A

factor IX

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3
Q

What type of inheritance pattern is exhibited by Hemophilia A and B?

A

X-linked

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4
Q

Factor activity for severe hemophiliacs and complications

A

<1%
spontaneous bleeds into joints and deep tissue
require factor replacement on ongoing basis

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5
Q

Factor activity and complications in moderate hemophiliacs.

A

1-5%

similar bleeds, but more often precipitated by minor trauma

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6
Q

factor activity and complications in mild hemophiliacs.

A

> 5%

may be asymptomatic until challenged by significant trauma or injury

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7
Q

Describe factor XI deficiency.

A

somewhat variable, mild bleeding tendency

relatively present in Ashkenazi Jew population

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8
Q

Describe factor XIII deficiency.

A

rare condition
classically presents with umbilical stump bleeding in the neonate
associated with defective wound healing and spontaneous abortion

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9
Q

two types of deficiencies in von Willebrand Disease

A

quantitative or qualitative

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10
Q

What factor is associated with vWF?

A

factor VIII

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11
Q

what type of bleeding is common in von Willebrand Disease?

A

mucocutaneous bleeding
(bleeding is usually mild to moderate)
soft tissue/joint bleeding in more severe cases

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12
Q

Inheritance pattern of most common type of von Willebrand disease.

A

autosomal dominant

characterized by a quantitative decrease in vWF

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13
Q

which type of vWD is characterized by quantitative reduction?

A

type I vWD

treated with desmopressin which stimulates release of vWF from endothelial cells

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14
Q

which type of vWD is characterized by qualitative, abnormal vWF protein?

A

type II vWD

treated with replacement therapy

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15
Q

What receptor is affected by Glanzmann’s Thrombasthenia?

A

Gp IIb/IIIa

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16
Q

what is main problem in Glanzmann’s Thrombasthenia?

A

defective platelet aggregation

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17
Q

What receptor is affected by Bernard-Soulier syndrome?

A

Gp Ib V-IX

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18
Q

what is main problem in Bernard-Soulier syndrome?

A

defective vWF-dependent adhesion by platelets

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19
Q

What is the most common cause of isolated thrombocytopenia in otherwise healthy individuals?

A

Idiopathic (autoimmune) thrombocytopenic purpura (ITP)

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20
Q

What characterizes idiopathic (autoimmune) thrombocytopenic purpura (ITP)?

A

anti-platelet antibodies, often directed against GP IIb/IIIa complexes, that result in antibody and Fc dependent platelet destruction (primarily in spleen)

21
Q

What type of autoantibody can cross the placenta?

A

IgG

22
Q

What types of drugs most commonly can cause drug-induced immune thrombocytopenia?

A

antibiotics (penicillin or sulfonamide-related), quinine compounds

23
Q

What should be stopped in the evaluation of thrombocytopenia?

A

all non-essential drugs

24
Q

What defines microangiopathic hemolytic anemia (MAHA)?

A

presence of RBC fragmentation (schistocytes) on the blood smear, varying degrees of anemia, elevated LDH, usually an elevated retic count

25
Q

When coagulopathy is present along with MAHA, what condition does it suggest?

A

disseminated intravascular coagulation (DIC)

26
Q

What protein becomes severely deficient in thrombotic thrombocytopenic purpura (TTP)?

A

ADAMTS13

27
Q

What is the function of ADAMTS13?

A

it is a vWF cleaving protease that prevents formation of large vWF multimers that partially unfold and bind to platelets

28
Q

What characterizes TTP?

A

MAHA, thrombocytopenia, fever, dysfunction of various organs (particularly brain (change in mental status) and kidneys)

29
Q

Is coagulopathy affected in TTP?

A

No, there is no consumption of clotting factors

30
Q

Treatments for TTP?

A

plasma exchange or infusion

steroids

31
Q

What organ is mostly involved in hemolytic-uremic syndrome (HUS)?

A

kidneys

32
Q

Classic symptom in hemolytic-uremic syndrome (HUS)

A

bloody diarrhea

33
Q

What type of infection is commonly found in hemolytic uremic syndrome (HUS)?

A

enterohemorrhagic bacteria expressing Shiga-like toxin (E. coli)
triggers endothelial injury

34
Q

Can idiopathic forms of HUS occur?

A

yes

35
Q

What enzyme synthesis is blocked by aspirin and NSAIDs?

A

Thromboxane

36
Q

What pathway does aspirin and NASAID block?

A

Cox-1

37
Q

describe effect of clopidogrel on platelet activation.

A

clopidorgrel inhibits ADP-mediated platelet activation

38
Q

4 causes of thrombocytosis

A

reactive
myeloproliferative disorders
post-splenectomy or asplenia
iron-deficiency

39
Q

What is the effect of Vitamin K deficiency on both PT and aPTT?

A

both are prolonged

40
Q

describe Hemorrhagic disease of the newborn (HDN)

A

newborn babies are vulnerable to bleeding due to vitamin K deficiency
there is poor transport of Vitamin K across placenta

41
Q

What is done to reduce risk of Vitamin K in infants?

A

all receive prophylactic Vitamin K

42
Q

Why can liver disease cause coagulopathy?

A

many of the coagulation factors are made in the liver

43
Q

What occurs in disseminated intravascular coagulation (DIC)?

A

thrombin and plasmin are generated at a rate that exceeds the ability of their natural inhibitors (antithrombin and antiplasmin) to neutralize them

44
Q

What usually causes DIC?

A

exposure to excessive amounts of Tissue Factor

45
Q

What problems are caused by DIC?

A

consumption of platelets (thrombocytopenia), depletion of inhibitors, bleeding, deposition of fibrin in small vessels with resulting microangiopathy, varying degrees of organ dysfunction

46
Q

Common lab findings in DIC

A

thrombocytopenia
Prolonged PT and aPTT
elevated FDP (fibrin degradation product) and D-dimer
decreased fibrinogen
red cell fragmentation on the blood smear

47
Q

How can you bypass inhibitors to factor VIII in treatment?

A

give factor VIIa

48
Q

What plays an important role in causing DIC?

A

diffuse endothelial injury

Hemostasis (1 decks)

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