[Fatal opportunistic infection following disappearance of antibodies by immunosuppressive therapy in a patient with acquired factor VIII inhibitor].
An 83-year-old man without history of the hemorrhagic diathesis was admitted to our hospital with a 4-months history of purpura and subcutaneous hematoma. He had an extraordinarily prolonged activated partial thromboplastin time, and his factor VIII (F VIII) activity level was 0.2%. A study revealed the existence of an IgG type anti-F VIII inhibitor at a titer of 1004 Bethesda units/ml. He received recombinant factor VIIa and immunosuppressive therapy with cyclophosphamide, prednisolone and cyclosporin, but despite this the titer of F VIII inhibitor remained high. Although the inhibitor disappeared after methylprednisolone mini-pulse therapy, the patient died of opportunistic infections with cytomegalovirus and pneumocystis carinii. The majority of patients with acquired F VIII inhibitor belong to the elderly population, and the standard therapeutic strategy to eliminate the acquired F VIII inhibitor has not been established. Those patients with high titers of F VIII inhibitor require particularly long term immunosuppressive therapy. Therefore, it is important to bear in mind treatment-related opportunistic infections in a case with a high titer of acquired F VIII inhibitor.
Kubota
Yasushi
Y
Division of Hematology, Department of Internal Medicine, Saga Medical School.
Sano
Masayuki
M
Nakazato
Sachie
S
Tsuneyoshi
Naoko
N
Tomimasu
Rika
R
Kuwahara
Nobuo
N
Fukushima
Noriyasu
N
Funai
Noriko
N
Suga
Kenji
K
Sueoka
Eisaburo
E
D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D003520:Cyclophosphamide / Q000009:adverse effects; D016572:Cyclosporine / Q000009:adverse effects; D003586:Cytomegalovirus Infections / Q000209:etiology*; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D017809:Fatal Outcome; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007166:Immunosuppressive Agents / Q000009:adverse effects*; D008297:Male; D009894:Opportunistic Infections / Q000209:etiology*; D016720:Pneumocystis Infections / Q000209:etiology*; D011239:Prednisolone / Q000009:adverse effects
D001323:Autoantibodies; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003520:Cyclophosphamide; D005169:Factor VIII; D011239:Prednisolone