Extramedullary haematopoietic tumor producing small intestinal intussusception in a beta-thalassemia/hemoglobin E Thai boy: a case report.

The authors report a case of beta-thalassemia/hemoglobin E disease with extramedullary haematopoietic tumor which developed at the small intestine and caused intussusception. A 7 year-old boy with homozygous beta-thalassemia/hemoglobin E presented with recurrent abdominal pain. The abdominal ultrasonography showed ileo-ileal intussusception with a solid mass as the leading point. Resection of the ileal segment was performed. Pathological examination revealed an extramedullary haematopoietic tumor forming an intraluminal polypoid mass, being the leading point of the intussusception. Extramedullary haematopoiesis in the intestinal tract is rare. To our knowledge, this is the first case of extramedullary haematopoietic tumor that produced intussusception of the small intestine in a beta-thalassemia/hemoglobin E patient.

Wilms' tumor, male pseudohermaphroditism and glomerulonephritis: the Drash syndrome. First case report in Thailand and review the literatures.

The Drash syndrome, the first case in Thailand is reported. The patient had male pseudohermaphrodite, Wilms' tumor of the left kidney and mesangial proliferative glomerulonephritis. Metastasis of Wilms' tumor was noted in the liver, omentum, pelvic peritoneum and vertebral body. Chemotherapy (actinomycin D, vincristine and dexamethasone) and local irradiation were given. The patient developed pneumonia, diarrhea and ended up with Steven-Johnson syndrome from Cotrimoxazole hypersensitivity.

Metastatic bone tumors in Ramathibodi Hospital, Thailand during 1985-90.

The study was aimed to analyse the histologic typings of metastatic bone tumors, age of patients and skeletal distributions in Ramathibodi Hospital. There were 122 cases of metastatic bone tumors out of 217 cases of all malignant bone tumors as retrieved from the files of surgical records in the Department of Pathology, Ramathibodi Hospital from 1985 to 1990. Approximately 80 per cent of metastatic bone tumors were carcinomas of which adenocarcinoma was the most common and accounted for 44.3 per cent of all metastatic bone tumors. The most common site of biopsy which possibly reflects the skeletal distribution of metastases was the vertebra which accounted for 35 per cent. The average age of patients presenting with metastatic carcinomas was about 50 years which was comparable to those of other series. It was obvious that biopsy of metastatic lesions was helpful in narrowing the scope of investigation but could hardly define the primary sites without further investigations.

Salmonella aortitis at Ramathibodi Hospital, Bangkok, Thailand.

Two cases of Salmonella aortitis were diagnosed by positive culture from the wall of aortic aneurysm. Common features in both cases included fever, abdominal/back pain and pulsatile abdominal mass with underlying severe atherosclerosis. The fatal outcome of both cases despite surgical and medical treatment was discussed and guideline of management was proposed.