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Flashcards in Bleeding disorders Deck (25):
1

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

mucosal bleeding: thrombocytopenia, defect in platelets or vWF

Deep tissue bleeding: defect in coagulation cascade

2

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

qualitative or quantitative

3

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

autosomal dominant

4

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

qualitative or functional defect, ie vWF type 2

5

How is type I vWD treated?

DDAVP: stimulates the release of endogenous vWF from endothelial cells

6

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

cryoprecipitate

7

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

amicar or tranexamic acid

8

Congenital deficiency of the __________ is known as Glanzmanns thrombasthenia

GpIIb/IIIa complex

characterized by deficient platelet aggregation in response to agonists

9

Congenital deficiency of ______ is known as Bernard-Soulier syndrome

GpIb

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

10

List diseases associated with decreased production of platelets

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

11

List three diseases associated with increased platelet utilization or destruction

immune mediated: ITP
non-immune mediated: DIC, TTP

12

What is the mechanism of ITP?

autoimmune destruction of platelets with normal or increased platelet production

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

13

Distinguish between ITP in children vs adults

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

In adults, more likely to be chronic and relapsing

14

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

neonatal thrombocytopenia

15

What agents are used to treat ITP?

glucocorticoids, IVIG, splenectomy, rituximab

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

16

What drugs most commonly cause drug induced immune thrombocytopenia

antibiotics, quinine

17

What two conditions are associated with microangiopathic hemolytic anemia?

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

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

uremia

19

List four causes of thrombocytosis

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

20

Newborns are vulnerable to bleeding due to _______ deficiency

vitamin K

21

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

Factors V, VII, IX, X, XI

treat with fresh frozen plasma

22

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

thrombin (inhibitor is antithrombin)
plasmin (inhibitor is a-2- antiplasmin)

23

DIC is a syndrome that can be associated with:

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

24

DIC involves diffuse endothelial injury. List laboratory findings in DIC

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

25

What patients are at risk of spontaneously developing coagulation inhibitors?

Hemophilia- directed against VIII
though autoantibodies can arise without pre-existing coagulation defect