Successful treatment of a patient with idiopathic factor VIII inhibitor with double filtration plasmapheresis and steroid administration.
We report a 74-year-old Japanese woman who had bleeding due to a factor VIII inhibitor in the absence of diseases known to be associated with its development. She abruptly developed a large painful purpura extending from the right hip to the thigh followed by an intramuscular haematoma of her left anterior chest. Examinations revealed a marked depression of factor VIII activity (5%) and the presence of 31 Bethesda units/ml factor VIII inhibiting activity. The phenotypes of these inhibitors were of the IgG-kappa and IgG-lambda types. She was treated with oral prednisolone and double filtration plasmapheresis (DFPP). The inhibitors rapidly disappeared after three sessions of plasmapheresis and her plasma factor VIII activity increased to a normal level. During this treatment, no major adverse effects such as thrombosis and infection were observed and transfusion of fresh frozen plasma (FFP) was not necessary. Heterogeneity of idiopathic factor VIII inhibitors in elderly patients is common and spontaneous disappearance or elimination of such inhibitors by treatment is often difficult to achieve. However, the combination of oral prednisolone and double filtration plasmapheresis is effective and safe for idiopathic factor VIII inhibitors and is a worthwhile approach to treatment.
Yamazaki
M
M
Department of Internal Medicine (III), Kanazawa University of Medicine, Japan.
Asakura
H
H
Jokaji
H
H
Saito
M
M
Uotani
C
C
Kumabashiri
I
I
Morishita
E
E
Nakamura
S
S
Naito
T
T
Ohta
H
H
D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology / Q000628:therapy*; D003131:Combined Modality Therapy; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D005260:Female; D006474:Hemorrhagic Disorders / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D010956:Plasmapheresis; D011239:Prednisolone / Q000627:therapeutic use*
D001323:Autoantibodies; D007074:Immunoglobulin G; D005169:Factor VIII; D011239:Prednisolone