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Split chimerism may be enough to cure Evans syndrome

Bone Marrow Transplantation volume 38, page 311 (2006)Cite this article

Evans 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 five 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.5, 6, 7, 8

A 20 month-old boy presented with fever, anemia, bruising, petechiae and splenomegaly. Investigations showed: RBC 2 420 000/μl, MCV 84 fl, reticulocytes 366 000/μl, WBC 3780/μl, neutrophils 47%, platelets 26 000/μl, direct Coombs test +++, LDH 2476, bilirubin 1.72 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.

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Authors and Affiliations

  1. Pediatric Hematology-Oncology, Ospedale dei Bambini ‘G. Di Cristina’, Palermo, Italy

    D Caselli, P Farruggia, A Trizzino, S Cannella, A Santoro & M Aricò

  2. Hematology I, A.O. Cervello, Palermo, Italy

    A Santoro

  3. Pediatric Hematology-Oncology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy

    F Locatelli

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  1. D Caselli
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  2. P Farruggia
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  7. M Aricò
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Caselli, D., Farruggia, P., Trizzino, A. et al. Split chimerism may be enough to cure Evans syndrome. Bone Marrow Transplant 38, 311 (2006). https://doi.org/10.1038/sj.bmt.1705427

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