Acute renal failure as a complication of acquired hemophilia due to autoantibody to factor VIII.
A 53-year-old Japanese woman, without any specific medical or family history, was admitted to our hospital for acute renal failure with macrohematuria. Routine blood analysis, including blood coagulation test, revealed azotemia accompanied by prolonged activated partial thromboplastin time (aPTT). Computed tomography revealed bilateral kidney swelling with dilatation of the renal pelvis. An extensive coagulation analysis revealed that the concentration of factor VIII had decreased to 1.8% and the level of factor VIII inhibitor was markedly elevated to 19 BU/ml. The final diagnosis was acquired hemophilia induced by autoantibodies against factor VIII, which was complicated by postrenal acute renal failure due to the obstruction of urinary tracts by renal bleeding and clots. The patient was treated with a combination of prednisolone at a dose of 50 mg/day (1 mg/kg body weight) and cyclophosphamide. The levels of factor VIII inhibitor decreased gradually, and the activity of factor VIII was improved after treatment. The levels of aPTT and concentrations of factor VIII and factor VIII inhibitor were monitored during the subsequent follow-ups.
Otaki
Yasuhiro
Y
Division of Clinical Nephrology and Rheumatology, Division of Cell Biology, Niigata University Graduate School of Medical and Dental Sciences, 1-757Asahimachi-dori, Niigata, 951-8510, Japan. kyoutei1gou@v102.vaio.ne.jp
Kouda
Ryo
R
Fujimura
Takeo
T
Nakatsue
Takeshi
T
Wakasugi
Minako
M
Murakami
Shuichi
S
Kuroda
Takeshi
T
Narita
Ichiei
I
Nakano
Masaaki
M
Gejyo
Fumitake
F
D058186:Acute Kidney Injury / Q000188:drug therapy / Q000209:etiology*; D001323:Autoantibodies / Q000097:blood*; D001778:Blood Coagulation Disorders / Q000175:diagnosis; D003520:Cyclophosphamide / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology*; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis* / Q000276:immunology; D006801:Humans; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D011239:Prednisolone / Q000627:therapeutic use; D016896:Treatment Outcome; D014570:Urologic Diseases / Q000150:complications
D001323:Autoantibodies; D003520:Cyclophosphamide; D005169:Factor VIII; D011239:Prednisolone