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Bone Marrow Transplantation (2006) 38, 311
& 2006 Nature Publishing Group All rights reserved 0268-3369/06 $30.00www.nature.com/bmtLETTER TO THE EDITORSplit chimerism may be enough to cure Evans syndromeBone Marrow Transplantation (2006) 38, 311.
doi:10.1038/sj.bmt.1705427; published online 19 June 2006Evans syndrome (ES) is a combination of autoimmune
hemolytic anemia (AIHA) and immune thrombocytopenia
(ITP).1 Treatment options include intravenous immunoglobulins (IVIG), corticosteroids, cyclosporine, vincristine,
danazol, azathioprine, cyclophosphamide, plasmapheresis,
rituximab and splenectomy.2,3 Only one-third of patients
were free of disease at 3 years after a median of ve
modalities of therapy in a recent survey, with seven dead,
12% with active disease on treatment, and 12% with
persistent disease.4 Hematopoietic stem cell transplant
(HSCT) has been used in a few cases of refractory ES.58A 20 month-old boy presented with fever, anemia,
bruising, petechiae and splenomegaly. Investigations
showed: RBC 2 420 000/ml, MCV 84 , reticulocytes
366 000/ml, WBC 3780/ml, neutrophils 47%, platelets
26 000/ml, direct Coombs test , LDH 2476, bilirubin1.72 mg/dl, AST 91, ALT 17. No autoantibodies (ANA,
ASMA, LKM) were found. Bone marrow was normocellular with erythroid hyperplasia, and normal myeloid
and platelet precursors.There was no response to prednisolone (20 mg/kg per
day; days 13)....