What is Coagulation Factor Inhibitor Related Disorder of Hemostasis?

Coagulation Factor Inhibitor Related Disorder of Hemostasis
Comparison of FXII−/− and FXI−/− mice in the tMCAO model. (A) Representative TTC-stained coronal sections of FXI−/− and WT mice. Infarct volumes were assessed 24 h after tMCAO (WT, n = 18 and FXI−/−, n = 11; *P < 0.05). (B) Accumulation of fibrin in the infarcted (+) and contralateral (−) hemispheres of WT, FXI−/−, and FXII−/− mice. Fibrin formation 24 h after ischemia was analyzed by immunoblotting using the fibrin-specific antibody 59D8. (C) Immunohistochemical colocalization of fibrin (59D8 antibody) and platelets (anti-GP Ib antibody) in the lumens of microvessels after 24 h in the infarcted hemisphere of WT mice. Bar, 100 μm. (D) Hematoxylin and eosin–stained sections of corresponding territories in the ischemic hemispheres of WT, FXI−/−, and FXII−/− mice. The dark arrows indicate blood vessels that are shown magnified in the inserts. Note the reduced microvascular patency in the ischemic areas of WT animals compared with FXI−/− and FXII−/− mice. Bar, 25 μm. The asterisk denotes the ischemic lesion. Targeting coagulation factor XII provides protection from pathological thrombosis in cerebral ischemia without interfering with hemostasis. Kleinschnitz C, Stoll G, Bendszus M, Schuh K, Pauer HU, Burfeind P, Renné C, Gailani D, Nieswandt B, Renné T - The Journal of experimental medicine (2006). Not Altered. CC.

Coagulation factor inhibitor related disorder of hemostasis are antibodies that either inhibit the activity or increase the clearance of a clotting factor.

The pathology of coagulation factor inhibitor-related disorder of hemostasis is:

-Etiology: The cause of coagulation factor inhibitor-related disorder of hemostasis is immune-mediated depletion or inhibition of a coagulation factor.

-Genes involved: None.

-Pathogenesis: The sequence of events that lead to coagulation factor inhibitor-related disorder of hemostasis shows that  specific or non-specific autoantibodies generate immune complexes with vWF, increasing its clearance from the circulation.

Patients with coagulation factor inhibitor-related disorder of hemostasis typically affect males and females both present at the age range of 26-45 years. The symptoms, features, and clinical findings associated with coagulation factor inhibitor-related disorder of hemostasis include hemorrhage, bleeding disorders, and bleeding diathesis.

Coagulation factor inhibitor-related disorder of hemostasis is diagnosed by doing a complete blood count. 

Coagulation factor inhibitor-related disorder of hemostasis is treated with  DDAVP, FVIII infusions, and bypassing agents.

The prognosis of coagulation factor inhibitor-related disorder of hemostasis is fair if treated properly in a timely manner.

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Last updated on May 24, 2022