A Case of Fatal Agranulocytosis That Developed in a Patient with ß-Thalassemia Major Treated with Deferiprone.

A 29-year-old male with transfusion-dependent ß-thalassemia major (ß-TM), splenectomized and on chelation therapy with deferiprone (DFP or L1) due to heart and liver hemosiderosis, presented with high fever and agranulocytosis. Deferiprone was discontinued and a broad spectrum antibiotic therapy was started intravenously. The patient remained febrile and showed no recovery of neutrophil count even after the initiation of granulocyte colony-stimulation factor (G-CSF). After 12 days at the hospital, he developed respiratory failure and was transferred to the intensive care unit (ICU) where he developed multi-organ failure and died 3 days later. To investigate the mechanism of agranulocytosis, bone marrow mononuclear cells of a healthy volunteer were plated on culture dishes, with or without the patient's serum. The observation of colony forming units of progenitor cells in dishes that contained the patient's serum, provided inconclusive explanation of the possible mechanism of DFP-induced agranulocytosis. This is a case of fatal agranulocytosis developing in a patient being treated with DFP, a well recognized but rare complication of this drug. Further studies are required in order to elucidate the possible pathogenic mechanism of agranulocytosis due to DFP and to provide clear guidelines in order to best care for the patient.

Gangrenous cystitis.

Gangrenous cystitis is an extremely rare condition. During the last 70 years, only 30 cases have been reported in the literature. We report a case of gangrenous cystitis in a 70-year-old woman presented with symptoms of acute abdomen. Main causative factor was overdistension of the bladder due to catheter obstruction. She underwent debridement and drainage of the cystic remnant. The pathogenesis, diagnosis, and management of gangrenous cystitis are discussed.

Limy bile syndrome: review of seven cases.

BACKGROUND: Milk of calcium bile or limy bile is a rare disorder in which the gall bladder is filled with a thick, paste-like, radiopaque material. METHODS: Seven patients with limy bile syndrome were treated in our department from 1980 to 2003. There were five women and two men, and their age ranged from 30 to 64 years. A retrospective analysis of clinical symptoms, diagnostic work-up, treatment approach and operative findings was performed. RESULTS: All patients presented with intermittent right upper abdominal quadrant pain. Three of the seven patients (42.85%) presented with complications like acute cholecystitis (two of seven patients) and obstructive jaundice (one of seven patients). Diagnosis was based on clinical findings, plain abdominal X-rays, ultrasonography and computed tomography scanning. Surgery was the treatment of choice and cholecystectomy alone or in combination with common bile duct exploration and drainage (if needed) was performed. CONCLUSION: The clinical aspect of the disease is similar to that of biliary lithiasis and the diagnosis is easily made by the characteristic spontaneous opacification of the gall bladder on plain abdominal X-rays. Complications such as acute cholecystitis, pancreatitis or obstructive jaundice can also be present. Although some cases of conservative pharmaceutical treatment as well as cases of spontaneous disappearance of limy bile have been reported, surgical treatment remains the treatment of choice.