A Bladder Hematoma Mimicking a Tumor in a Newborn with Severe Hemophilia A

Patients with hemophilia A (OMIM 306700), an inherited X-linked recessive disease, present with increased bleeding symptoms because of deficiency or complete absence of factor VIII. The residual factor VIII activity determines the severity and frequency of bleeding episodes and the manifestation as mild, moderate and severe hemophilia A. Usually, patients with severe hemophilia A display spontaneous joint or deep muscle bleeding. Patients with a moderate phenotype present with seldom bleeding episodes and those with a mild phenotype hardly show any abnormal bleeding, until encountered with trauma or surgery. Patients with hemophilia may also rarely present with a pseudo-tumor/-cyst, which could form due to repetitive bleeding, clot accumulation and necrotic tissue at the site of bleeding (reviewed in Rodriguez-Merchan EC, Haemophilia 2002; 8: 393–401). In several instances, surgical intervention has been necessary to control these hemophilic pseudocysts.