Bleeding disorders Flashcards

1
Q

In general, mucosal bleeding is suggestive of _____ or a defect in ______ while deep tissue bleeding is suggestive of a defect in _______

A

mucosal bleeding: thrombocytopenia, defect in platelets or vWF

Deep tissue bleeding: defect in coagulation cascade

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2
Q

von Willebrand disease is a _____ or _____ deficiency in vWF, usually with a parallel decrease in factor VIII function

A

qualitative or quantitative

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3
Q

The most common form of vWD is inherited in _______ manner and characterized by a quantitative decrease in vWF

A

autosomal dominant

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4
Q

If there is a significant disparity between vWF antigen and activity, this suggests a _______ defect in vWF

A

qualitative or functional defect, ie vWF type 2

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5
Q

How is type I vWD treated?

A

DDAVP: stimulates the release of endogenous vWF from endothelial cells

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6
Q

_______ contains a large amount of vWF and can be given in sever vWF deficiency

A

cryoprecipitate

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7
Q

Antifibrinolytic agents such as ______ or ____ can be useful for mucocutaneous bleeding in patients with vWD

A

amicar or tranexamic acid

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8
Q

Congenital deficiency of the __________ is known as Glanzmanns thrombasthenia

A

GpIIb/IIIa complex

characterized by deficient platelet aggregation in response to agonists

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9
Q

Congenital deficiency of ______ is known as Bernard-Soulier syndrome

A

GpIb

results in platelets with defective vWF dependent adhesion, usually mild thrombocytopenia with large circulating platelets

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10
Q

List diseases associated with decreased production of platelets

A
bone marrow disorders ex leukemia
MDS
aplastic anemia
cytotoxic drugs
viral infection
nutritional deficiency
marrow fibrosis
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11
Q

List three diseases associated with increased platelet utilization or destruction

A

immune mediated: ITP

non-immune mediated: DIC, TTP

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12
Q

What is the mechanism of ITP?

A

autoimmune destruction of platelets with normal or increased platelet production

usually antibodies against GpIb or GpIIb/IIIa, leads to platelet destruction in the spleen

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13
Q

Distinguish between ITP in children vs adults

A

In children, usually acute form due to infection and resolves spontaneously

In adults, more likely to be chronic and relapsing

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14
Q

In pregnant women with ITP, IgG can cross the placenta and occasionally cause:

A

neonatal thrombocytopenia

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15
Q

What agents are used to treat ITP?

A

glucocorticoids, IVIG, splenectomy, rituximab

new agents: eltrombopag and romiplostim are TPO agonists, stimulate platelet production

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16
Q

What drugs most commonly cause drug induced immune thrombocytopenia

A

antibiotics, quinine

17
Q

What two conditions are associated with microangiopathic hemolytic anemia?

A

TTP: deficiency of ADAMTS-13, results in deposition of vWF thrombi in blood vessels leading to microangiopathy

HUS: kidneys are predominant organ affected, shiga toxin triggers endothelial injury leading to microangiopathy

18
Q

_____ and drugs like NSAIDS and aspirin are associated with acquired defects in platelet function

A

uremia

19
Q

List four causes of thrombocytosis

A

reactive- infection or trauma
myeloproliferative disorder
post-splenectomy or asplenia
iron deficiency

20
Q

Newborns are vulnerable to bleeding due to _______ deficiency

A

vitamin K

21
Q

Sever liver disease causes decreased synthesis of fibrinogen, prothrombin, factors _____________. Bleeding can be treated with replacement therapy with ______

A

Factors V, VII, IX, X, XI

treat with fresh frozen plasma

22
Q

DIC is a syndrome in which ____ and _____ are generated at a rate that exceeds the ability of their natural inhibitors to neutralize them

A

thrombin (inhibitor is antithrombin)

plasmin (inhibitor is a-2- antiplasmin)

23
Q

DIC is a syndrome that can be associated with:

A
infection
tumors, esp adenocarcinoma
leukemia
obstetric complications
hemolytic transfusion reactions
liver disease
trauma
shock
surgery
24
Q

DIC involves diffuse endothelial injury. List laboratory findings in DIC

A
thrombocytopenia
Prolonged PT/INR and aPTT
Elevated FDP and D dimer
Decreased fibrinogen
Red cell fragmentation on blood smear
25
Q

What patients are at risk of spontaneously developing coagulation inhibitors?

A

Hemophilia- directed against VIII

though autoantibodies can arise without pre-existing coagulation defect