Acquired hemophilia: a case report of 2 patients with acquired factor VIII inhibitor treated with rituximab plus a short course of steroid and review of the literature.
Acquired hemophilia is an unusual disorder in which nonhemophiliac patients develop autoantibodies (inhibitor) against the factor VIII coagulation protein. Factor VIII inhibitor leads to life-threatening bleeding disorders classically described as new onset of diffuse bruising and prolonged partial thromboplastin time in elderly patients. Treatment is focused in the control of the acute bleeding episode and the long-term suppression of the autoantibody. Several immunosuppressive combinations have been described; however, these treatments are also associated with serious side effects that are difficult to tolerate, especially in older and debilitated patients. New treatment modalities explore the elimination of the autoantibody production by targeting B-cells with rituximab, an anti CD-20 monoclonal antibody that has shown success in a multitude of autoimmune processes. This report presents 2 patients successfully treated with rituximab and a short tapering course of steroids and focuses our discussion in the analysis of different treatment approaches available for these patients' population.
Alvarado
Yesid
Y
Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas 79403, USA.
Yao
Xin
X
Jumper
Cynthia
C
Hardwicke
Fred
F
D'Cunha
Nicholas
N
Cobos
Everardo
E
D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000628:therapy*; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology*; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D000069283:Rituximab; D013256:Steroids / Q000627:therapeutic use
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab; D005169:Factor VIII