Successful treatment of thrombocytopenia and hemolytic anemia with IvIG ina patient with lupus-like syndrome after mismatched related PBSCT

Hematopoietic stem cell transplantation (HSCT) is a treatment option for au toimmune diseases but can also cause clinical features similar to those of autoimmune diseases. In some of these cases the autoimmune-like condition i s associated with autoimmune cytopenia, a complication that can be unrespon sive to established treatment strategies and which may be fatal. The majori ty of cases reported on immune hemolytic anemia have been of alloimmune ori gin due to ABO red blood cell antigen incompatibilities between donor and r ecipient. We now report a patient with a lupus-like syndrome, presenting wi th severe thrombocytopenia and hemolytic anemia 9 months after HLA-mismatch , ABO compatible-related PBSCT who experienced no response to high-dose ste roids, but who had a sustained response to repeated IvIG therapy.