Long-lasting remission and successful treatment of acquired factor VIII inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus.
Acquired deficiency of clotting factor VIII (FVIII) is a rare bleeding diathesis seldom encountered in systemic lupus erythematosus (SLE). Reduction of FVIII activity by autoantibodies can cause potentially life-threatening situations. Herein, an SLE patient with a positive lupus anticoagulant (LAC) test who abruptly developed metrorrhagia 4 yr after diagnosis is reported. Coagulation tests revealed FVIII activity reduced to 3% and a prolonged aPTT. FVIII inhibitor(s) were found to be as high as 3.0 Bethesda Units. Plasmapheresis, immunoglobulins, prednisolone and FVIII plasma concentrates induced the cessation of metrorrhagia, but the clotting tests were barely improved. One month later, extensive ecchymosis appeared and worsened, despite re-administration of the previous therapy. Pulse cyclophosphamide followed by oral administration was then started with normalization of coagulation parameters and long-lasting disease remission.
Trotta
F
F
Division of Rheumatology, Arcispedale S. Anna, University of Ferrara, Italy.
Bajocchi
G
G
La Corte
R
R
Moratelli
S
S
Sun
L Y
LY
D000284:Administration, Oral; D000328:Adult; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005169:Factor VIII / Q000276:immunology*; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008796:Metrorrhagia / Q000209:etiology; D020551:Pulse Therapy, Drug; D012074:Remission Induction
D007166:Immunosuppressive Agents; D016682:Lupus Coagulation Inhibitor; D003520:Cyclophosphamide; D005169:Factor VIII