Autoanti-Factor VIII Inhibitor and Acquired Hemophilia - Disseminated Intravascular Coagulation

Question 1

Specifically inhibit factors II (pro-thrombin), V, VIII, IX, and XIII and VWF have been described in nonhemophilic patients

Answer 1

Acquired autoantibodies

Question 2

The most common auto-antifactor which is a diagnostic of acquired hemophilia

Answer 2

Factor VIII

Question 3

Ocasionally associated with rheumatoid arthritis, inflammatory bowel disease, systemic lupus erythematosus, or lymphoproliferative disease

Answer 3

Acquired hemophilia

Question 4

Lab result in Acquire Hemophilia:
PTT:
PT and TT:

Answer 4

PTT: Prolonged
PT and TT: Normal

Question 5

This test confirm the presence of the inhibitor.

Answer 5

Clot-based mixing studies

Question 6

The return of prolongation after incubation occurs because factor VIII autoantibodies are often of what immunoglobulin isotype?

Answer 6

G4 isotype

Question 7

This inhibitors may immediately prolong the mixture’s PTT; in this case an incubated mixing study is unnecessary

Answer 7

High avidity inhibitors

Question 8

Develop in 30% of patients with severe hemophilia in response to factor VIII concentrate therapy, exhibit type I kinetics.

Answer 8

Alloantibodies to Factor VIII

Question 9

There is linear in vitro neutralization of factor VIII activity over 2 hours, which results in complete inactivation

Answer 9

Type 1 kinetics

Question 10

T/F: Type II kinetics, the titration of inhibitor activity is semiquantitative

Answer 10

TRUE

Question 11

Quantitation of autoanti-VIII inhibitor is accomplished using the __ which is ordinarily employed to measure inhibitors in hemophilic patients with alloantibodies to factor VIII

Answer 11

Nijmegen-Bethesda assay

Question 12

Are used to follow the response to immunosuppressive therapy but are not needed for management of the bleeding symptoms

Answer 12

Repeat titers

Question 13

Antibodies that prolong the PT

Answer 13

Antiprothrombin (Factor II) antibodies

Question 14

Autoantibodies to factor V may arise spontaneously in auto-immune disorders and after exposure to

Answer 14

Bovine thrombin fibrin glue

Question 15

Deficiency in amyloidosis may be caused by what seems to be an absorptive mechanism

Answer 15

Autoanti-factor X

Question 16

This may bypass the coagulation factor VIII inhibitor in acquired hemophilia and thereby control acute bleeding

Answer 16

Activated PCC or rFVIIa

Question 17

Deficiency with symptoms similar to those of congenital VWD, has been described in hypothyroidism, benign monoclonal gammopathies, Wilms tumor, intestinal angiodysplasia, congenital heart disease, pesticide exposure, uremia, lupus erythematosus, and autoimmune, lymphoproliferative, and myeloproliferative disorders.

Answer 17

Acquired vWF Deficiency

Question 18

Lab result in Acquired vWD Deficiency
PT:
PTT:
vWF:

Answer 18

PT: Not affected
PTT: Moderately prolonged
vWF: Reduced

Question 19

No longer recommended for treatment of VWD because it does not undergo viral inactivation.

Answer 19

Cryoprecipitate