Acute autoimmune hemolytic anemia following unrelated cord blood transplantation as an early manifestation of chronic graft-versus-host disease

A 16-month-old girl diagnosed with osteopetrosis underwent an unrelated, pa rtially matched (with major mismatch at A locus) cord blood stem cell trans plant. Twelve months later she developed severe acute autoimmune hemolytic anemia (AIHA). Immunophenotype analysis of lymphocyte subsets 8 months post transplant showed a low number of T lymphocytes, with normal subsets, and with NK cells and B lymphocytes within normal ranges. When the hemolytic an emia developed, the lymphocytes subsets changed and analysis showed higher numbers of B lymphocytes than previously, lower CD3(+) T lymphocytes with i nversion of the CD4/CD8 ratio and an abnormal proportion of T lymphocyte su bsets. She was being treated with cyclosporine, and steroids and immunoglob ulins were added. Initially the AIHA improved, but repeated infectious epis odes led us to tail off the immunosuppressive treatment. The AIHA relapsed and cyclosporine was restarted. Currently, she is on cyclosporine and low-d ose steroid treatment with no hemolytic features. During the 3 months when the AIHA was being treated, she developed extensive skin cGVHD and recurren t pneumothoraces. AIHA may be the first manifestation of abnormal reconstit ution of immunity developing after a hematopoietic transplant. This abnorma l reconstitution is also the basis of cGVHD. We suggest that aggressive imm unosuppressive treatment with intensive measures against infection could gi ve a better prognosis to such patients.