Hemostasis Disorders

Front 1

____________ is the formation of a weak platelet plug and ___________ is the stabilization of that plug.

Back 1

Primary hemostasis is formation of weak platelet plug; secondary hemostasis is turning platelet plug into a strong clot plug.

Front 2

Primary hemostasis is due to what general abnormality:
A. platelet abnormalities
B. coagulation cascade

Back 2

A. platelet abnormalities

Front 3

Immune Thrombocytopenic Purpura (ITP) is:
A. autoimmune production of IgG against platelet antigens (ex. GPIIb/IIIa)
B. pathologic formation of platelet microthrombi in small vessels
C. microthrombi in small vessels due to uncleaved large vWF multimers
D. microthrombi in small vessels due to endothelial damage by drugs or infection

Back 3

A. autoimmune production of IgG against platelet antigens (ex. GPIIb/IIIa

Autoantibodies are produced by plasma cells in the spleen; antibody bound platelets are consumed by splenic macrophages, resulting in thrombocytopenia.

Front 4

autoimmune production of IgG against platelet antigens (ex. GPIIb/IIIa)

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 4

A. Immune Thrombocytopenic Purpura (ITP)

Front 5

pathologic formation of platelet microthrombi in small vessels

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 5

B. Microangiopathic Hemolytic Anemia

Front 6

microthrombi in small vessels due to uncleaved large vWF multimers

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 6

C. Thrombotic Thrombocytopenic Purpura (TTP)

b/c of decreased ADAMTS13. ADAMTS13 is an enzyme that normally cleaves vWF multimers into smaller monomers for eventual degradation. Large uncleaved multimers lead to abnormal platelet adhesion, resulting in microthrombi.

Front 7

microthrombi in small vessels due to endothelial damage by drugs or infection

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 7

D. Hemolytic uremic syndrome (HUS)

* due to endothhelial damage by drugs or infection, classically E. coli in children

Front 8

genetic defect of GPIb deficiency; adhesion is impaired

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 8

E. Bernard-Soulier syndrome

Front 9

genetic Giib/IIIa deficiency; aggregation is impaired

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 9

F. Glanzmann thrombasthenia

Front 10

MCC of thrombocytopenia in children AND adults

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 10

A. Immune Thrombocytopenic Purpura (ITP)

Autoantibodies are produced by plasma cells in the spleen; antibody bound platelets are consumed by splenic macrophages, resulting in thrombocytopenia.

Front 11

Autoantibodies are produced by plasma cells in the spleen; antibody bound platelets are consumed by splenic macrophages, resulting in thrombocytopenia.

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 11

A. Immune Thrombocytopenic Purpura (ITP)

Front 12

* see schistocytes (sheared RBC, hemolysis) aka "helmet cell"

A. Immune Thrombocytopenic Purpura (ITP)
B. Microangiopathic Hemolytic Anemia
C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)
E. Bernard-Soulier syndrome
F. Glanzmann thrombasthenia

Back 12

B. Microangiopathic Hemolytic Anemia

C. Thrombotic Thrombocytopenic Purpura (TTP)
D. Hemolytic uremic syndrome (HUS)

Front 13

Secondary hemostasis problem is do to
A. problem with the platelets
B. problem with coagulation cascade

Back 13

B. problem with coagulation cascade

Front 14

* deep tissue bleeding into muscle s and joints and rebleeding after surgical procedures is seen in :
A. primary hemostasis disorders
B. secondary hemostasis disorders

Back 14

B. secondary hemostasis disorders
like Hemophilia A and B, Coagulation Factor Inhibitor vW disease, and can also occur in Vit K deficiency, liver failure and large-volume transfusion.

Front 15

For which disease does the PTT NOT correct with the mixing study?
A. Hemophilia A
B. Hemophilia B
C. Coagulation Factor Inhibitor
D. von Willebrand's disease

Back 15

C. Coagulation Factor Inhibitor

Front 16

True or False:
von Willebrand's disease is an autosomal dominant condition.

Back 16

True

decreased vWF levels.

Front 17

Vitamin K deficiency prevents functional activation of which factors?

Back 17

Factor II, X, VII, IX

Front 18

With regards to von Willebrand's disease, which is considered a general qualitative defect?
A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 18

B. Type IIA

abnormal structure/function of vMF: abnormal synthesis or proteolysis

Front 19

With regards to von Willebrand's disease, which is associated with increased binding to platelets?

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 19

C. Type IIB

abnormal structure/function of vMF: enhanced binding to platelets by large multimers

Front 20

With regards to von Willebrand's disease, which is associated with decreased affinity for GPIb?

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 20

D. Type IIM

abnormal structure/function of vMF: reduced affinity for GP Ib-IX

Front 21

With regards to von Willebrand's disease, which is associated with decreased dffinity for Factor VIII?

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 21

E. Type IIN

abnormal structure/function of vMF: reduced affinity for factor VIII

Front 22

With regards to von Willebrand's disease, which is associated with Severe absence of vWF?

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 22

F. Type III

severe deficiency in sheer number of vWF

Front 23

With regards to von Willebrand's disease, which is associated with Factor VIII activity and antigen levels are reduced.

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 23

E. Type IIN

abnormal structure/function of vMF: reduced affinity for factor VIII

Front 24

With regards to von Willebrand's disease, which is associated with vWF antigen increased, RIPA increased.

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 24

C. Type IIB

abnormal structure/function of vMF: enhanced binding to platelets by large multimers

Front 25

With regards to von Willebrand's disease, which is associated with vWF antigen normal, vWF activity reduced, RIPA reduced.

A. Type I
B. Type IIA
C. Type IIB
D. Type IIM
E. Type IIN
F. Type III

Back 25

B. Type IIA

abnormal structure/function of vMF: abnormal synthesis or proteolysis

Front 26

What three things increase the amount of von Willebrand Factor released by the Weilberl-Palade bodies? (if you wanna milk em)

Back 26

Stress, hormones (esp estrogen), and vasopressin

Front 27

Not all hypercoaguable states are created equally. Realize that the venous risk factors are largely [ acquired / inherited ] and the arterial ones moreso [ acquired / inherited ] though some are the other. Note that a risk factor for both – the hyperhomocysteinemia- is actually very much so an inherited disease too.

Back 27

arterial are usually acquired (tho possibly inherited too) ; venous are largely inherited

Front 28

What are risk factors for BOTH arterial and venous hypercoagulable states? (6)

Back 28

rterial are usually acquired (tho possibly inherited too) ; venous are largely inherited. But for BOTH: hyperhomocysteinemia, antiphospholipid antibodies, hormone medications, cigarettes, MPD, PNH.