A Case of Acquired Hemophilia a Discovered by Right Renal Bleeding

Abstract

An 81-year-old man was referred to our hospital with a chief complaint of asymptomatic macrohematuria. Cystoscopy revealed bleeding from the right ureteral orifice. Various examinations, including ureteroscopy, failed to find any abnormalities. As gingival bleeding followed the macrochematuria, further examinations of blood coagulatory function were undertaken. Decreased factor VIII coagulant activity accompanied by the presence of factor VIII inhibitor was revealed, leading to a final diagnosis of acquired hemophilia A. Macrohematuria and gingival bleeding immediately disappeared with oral administration of prednisolone at 30 mg per day, and the titer of factor VIII inhibitor decreased to an undetectable level by the 45th day of treatment. We emphasized the importance of blood coagulation testing in the examination of patients with macrohematuria.

First identification and expression of a type 2N von Willebrand disease mutation (E1078K) located in exon 25 of von Willebrand factor gene

“…The R854Q mutation, in exon 20, is the most frequent mutation identified, on at least one allele, in 90% of type 2N VWD patients studied thanks to the French INSERM Network (unpublished data). Until now, only the Q1053H and C1060R mutations in exon 24 [2] and the C1225G mutation in exon 27 of VWF gene [3] have been identified outside the N-terminal FVIII binding domain of VWF.…”

“…Hematuria and renal-ureteral hemorrhage are common complications in patients with hemophilia and may be caused by trauma or spontaneous in nature [1,2]. The occurrence of a spontaneous dissecting intramural hemorrhage of the ureter is a rare event and so far has not been reported in the literature.…”

Acute renal failure as a complication of acquired hemophilia due to autoantibody to factor VIII

“…In AH patients, bleeding tends occur in soft tissue, muscles, retroperitoneal space, and gastrointestinal tracts, and sometimes the genitourinary tract [3]. Although there have been some reports on AH patients presenting with micro-or macrohematuria [4,5], there has been no report on AH patients presenting with acute renal failure who were treated with renal replacement therapy. Herein, we present a rare case of an AH patient presenting with acute renal failure, which resulted from the bilateral obstruction of the urinary tracts by coagulated blood.…”

Successful management of intramural ureteral hemorrhage in a patient with factor VIII deficiency and high‐titer inhibitor

“…Hematuria and renal‐ureteral hemorrhage are common complications in patients with hemophilia and may be caused by trauma or spontaneous in nature [1,2]. The occurrence of a spontaneous dissecting intramural hemorrhage of the ureter is a rare event and so far has not been reported in the literature.…”

“…This patient is unusual in his presentation of a seemingly spontaneous ureteral bleed that extended along the connective tissue smooth muscle layer of the ureter. Spontaneous ureteral hematomas have been described in only two previous case reports, one a patient with acquired hemophilia and in another patient with overdosage of heparin [2,3]. The finding of hemorrhage and fluid in the iliopsoas also has been described in the literature as a result of the ureteral hemorrhage allowing extravasation into the iliopsoas muscle [1].…”