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

1
Q

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

A

Acquired autoantibodies

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2
Q

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

A

Factor VIII

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3
Q

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

A

Acquired hemophilia

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4
Q

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

A

PTT: Prolonged
PT and TT: Normal

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5
Q

This test confirm the presence of the inhibitor.

A

Clot-based mixing studies

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6
Q

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

A

G4 isotype

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7
Q

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

A

High avidity inhibitors

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8
Q

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

A

Alloantibodies to Factor VIII

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9
Q

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

A

Type 1 kinetics

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10
Q

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

A

TRUE

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11
Q

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

A

Nijmegen-Bethesda assay

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12
Q

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

A

Repeat titers

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13
Q

Antibodies that prolong the PT

A

Antiprothrombin (Factor II) antibodies

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14
Q

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

A

Bovine thrombin fibrin glue

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15
Q

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

A

Autoanti-factor X

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16
Q

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

A

Activated PCC or rFVIIa

17
Q

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.

A

Acquired vWF Deficiency

18
Q

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

A

PT: Not affected
PTT: Moderately prolonged
vWF: Reduced

19
Q

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

A

Cryoprecipitate