Congenital dyserythropoietic anemia type II with a positive sucrose hemolysis test

Hemophagocytic syndrome (HPS) is a rare clinical presentation infrequently associated with lymphoproliferative disorders. We describe a 29-year-old male with aggressive HPS and T-cell lymphoma managed successfully with high-dose chemotherapy and autologous peripheral stem cell transplantation (APSCT), in remission at 41 months of follow-up. In reviewing the literature, this case illustrates the 2nd longest surviving individual post stem cell transplant for aggressive HPS.