Vesical varices and telangiectasias in a patient with ataxia telangiectasia.
A Japanese boy with ataxia telangiectasia (AT) developed severe gross hematuria and recurrent bladder tamponade, requiring an extensive blood transfusion. He had received intermittent intravenous cyclophosphamide pulse therapy (cumulative dose of 1.3 g) for refractory steroid-resistant and intravenous immunoglobulin-resistant severe autoimmune thrombocytopenia 3 years previously. A cystoscopy revealed multiple varices and severe telangiectasias in the bladder wall. The intensive treatment, such as repeatedly selective embolization of the vesical arteries, proved to be partially effective. Finally, a surgical cystotomy resulted in a gradual improvement in clinical symptoms. To the best of our knowledge, this is the first report of a patient with AT who developed refractory bladder hemorrhage caused by widespread vesical telangiectasias.
Suzuki
Koichi
K
Department of Pediatrics, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. skoichi@infoaomori.ne.jp
Tsugawa
Koji
K
Oki
Eishin
E
Morio
Tomohiro
T
Ito
Etsuro
E
Tanaka
Hiroshi
H
D000283:Administration, Intravesical; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000473:pathology / Q000628:therapy; D001803:Blood Transfusion; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003558:Cystoscopy; D004564:Electrocoagulation; D004621:Embolization, Therapeutic; D006417:Hematuria / Q000209:etiology; D006470:Hemorrhage / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D053306:Hyper-IgM Immunodeficiency Syndrome / Q000150:complications / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D020551:Pulse Therapy, Drug; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000188:drug therapy / Q000209:etiology; D012835:Silver Nitrate / Q000008:administration & dosage; D013684:Telangiectasis / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome; D001743:Urinary Bladder / Q000098:blood supply*; D001745:Urinary Bladder Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D013520:Urologic Surgical Procedures; D014648:Varicose Veins / Q000209:etiology* / Q000473:pathology / Q000628:therapy
D007166:Immunosuppressive Agents; D012835:Silver Nitrate