APLASTIC-ANEMIA IN EOSINOPHILIC FASCIITIS - RESPONSES TO IMMUNOSUPPRESSION AND MARROW TRANSPLANTATION

Eosinophilic fasciitis (EF) is a rare connective tissue disorder which is frequently associated with hematologic disorders, especially aplas tic anemia (AA) and variants (amegakaryocytic thrombocytopenia). The p rognosis for AA with EF has generally been poor, bur a few reports sug gest a role for immunosuppressive therapy. We have seen four cases of AA complicating EE All received corticosteroids and anti-thymocyte glo bulin without any benefit. One patient died of bleeding and infection. A second achieved unmaintained partial remission after two courses of cyclosporine A, although he had difficulty with side effects. Two pat ients received bone marrow transplants and both initially engrafted we ll. One had received marrow from a phenotypically HLA-matched parent a nd died of late graft failure. The second transplanted patient appears to be the only reported case of long term cure of both the AA and EE Our four patients constitute the largest reported series of AA with EF and shed light on clinical aspects of the disease, and on the pathoge nesis, particularly on responsiveness to different therapies; furtherm ore, there are implications to the treatment of AA in general.