title: Cyclophosphamide Treatment for Acquired Factor VIII Inhibitor in a Patient with AIDS-Associated Progressive Multifocal Leukoencephalopathy.
abstract: Acquired hemophilia A (AHA) is a severe bleeding disorder with high mortality rates resulting from the development of autoantibodies to factor VIII (FVIII). Patients typically present with hemorrhages in the skin, subcutaneous tissues, and muscles, which are frequently severe. They can also develop life-threatening retroperitoneal hematomas and compartment syndromes. We describe the case of a man with a long history of AIDS complicated by progressive multifocal leukoencephalopathy (PML), who developed AHA while on stable antiretroviral therapy and then presented with new onset bleeding and hypotension. We treated our patient with incrementally increasing doses of cyclophosphamide resulting in resolution of coagulopathy. We review the medical literature for additional cases of HIV-associated AHA and discuss the challenges in the care of our patient, since the immunosuppression needed to eradicate the FVIII inhibitor had the potential to cause recrudescence of his PML.
authors.affiliation: Divisions of Infectious Diseases, Virginia Mason Medical Center, Seattle, WA, USA Section of Hematology and Oncology, Virginia Mason Medical Center, Seattle, WA, USA.
authors.forename: Uma
authors.initials: U
authors.lastname: Malhotra
authors.affiliation: Divisions of Infectious Diseases, University of Washington, Seattle, WA, USA Hematology, University of Washington, Seattle, WA, USA hemdma@vmmc.org.
authors.forename: David M
authors.initials: DM
authors.lastname: Aboulafia
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000150:complications*; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005169:Factor VIII / Q000378:metabolism; D006401:Hematologic Agents / Q000008:administration & dosage*; D006467:Hemophilia A / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications*; D008297:Male; D008875:Middle Aged
chemical_list: D006401:Hematologic Agents; D003520:Cyclophosphamide; D005169:Factor VIII
keywords: AIDS-associated progressive multifocal leukoencephalopathy