The role of surgery in intraabdominal Burkitt's lymphoma in children.

The role of surgery in intraabdominal Burkitt's lymphoma remains controversial and different opinions are present in the literature. In our institution, forty patients (30 boys and 10 girls) with intraabdominal Burkitt's lymphoma with ages ranging from 3 to 12 years have been treated and followed from 1989 through 2000. In ten cases, the patients underwent surgery because of their acute abdominal diseases (intestinal obstruction in 5, intussusception in 3, intestinal perforation in one, and acute appendicitis in one). The remaining thirty patients were referred to our clinic because of their abdominal masses, pain, anorexia and fatigue. Twelve children had localized tumors and total resection could be performed. There was one death in this group due to central nervous system involvement during chemotherapy. In the remaining 28 children, extensive intraabdominal diseases were detected. In four of them, debulking procedures were performed, while in 24 children only biopsies could be made; 8 of them underwent a second-look operation. In the debulking procedures group, two children were lost (50 %) due to tumorlysis and acute renal failure. In the biopsy group, there were six deaths (25 %). All patients received chemotherapy after operative recovery. In conclusion, our results suggest that when the tumor is localized, total resection results in a good outcome. However, in the presence of extensive intraabdominal diseases instead of resection, the operation should be limited to biopsy only.     

Traumatic rupture of hydatid cyst with unusual localization without liver involvement: a case report.

Primary hydatid disease of the intestinal mesentery is rare. We report on a 12-year-old male patient who presented with abdominal pain and urticaria lesions after blunt abdominal trauma. At laparotomy the cyst was found to be ruptured and was totally removed from the surrounding mesenteric tissue.The postoperative course was uneventful. This case is the first child reported with traumatic rupture of a hydatid cyst in a mesenteric location.     

INTRA-AORTIC BALLOON RUPTURE CAUSING FEMORAL ENTRAPMENT

Rupture of an intra-aortic balloon counterpulsator (IABCP) demands immediate removal. We report a case of thrombus formation within a Datascope IABCP secondary to IABCP rupture, necessitating surgical exploration for removal. There is a disturbing pattern of balloon ruptures with this type of IABCP.     

PJA-BP expression and TCR delta deletion during human T cell differentiation

Recombination of deltaRec to psiJalpha will delete the TCR delta gene, which is thought to play an important role in the bifurcation of the TCR alphabeta versus TCR gammadelta differentiation lineages. We recently detected a DNA-binding protein in human thymocytes, the so- called PJA-BP, which recognizes the psiJalpha gene segment and might be one of the factors involved in the regulation of preferential deltaRec- psiJalpha rearrangements. We now investigate PJA-BP expression and its correlation with TCR delta gene deletion in thymocytes. Our electrophoretic mobility shift assay experiments showed that the PJA-BP is evolutionary conserved in human, murine and simian thymocytes. Using a large series of human hematopoietic malignancies (n = 30), we conclude that PJA-BP expression is thymocyte specific and seems to be restricted to thymocytes committed to the TCR alphabeta lineage. Analysis of seven well-defined human thymocyte subpopulations showed that preferential deltaRec-psiJalpha rearrangements as well as PJA-BP expression can be detected from the immature CD34-/CD1+/CD3- /CD4+/CD8alpha+beta- thymocyte differentiation stage onwards. These experiments indicate that expression of PJA-BP in human thymocytes starts simultaneously with preferential deltaRec-psiJalpha rearrangements, which supports our hypothesis that PJA-BP is one of the factors involved in the preferential recombination of deltaRec to psiJalpha.