Aplastic anemia in eosinophilic fasciitis: responses to immunosuppression and marrow transplantation.

Overview

Eosinophilic fasciitis (EF) is a rare connective tissue disorder which is frequently associated with hematologic disorders, especially aplastic anemia (AA) and variants (amegakaryocytic thrombocytopenia). The prognosis for AA with EF has generally been poor, but a few reports suggest a role for immunosuppressive therapy. We have seen four cases of AA complicating EF. All received corticosteroids and anti-thymocyte globulin 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 patients received bone marrow transplants and both initially engrafted well. One had received marrow from a phenotypically HLA-matched parent and 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 EF. Our four patients constitute the largest reported series of AA with EF and shed light on clinical aspects of the disease, and on the pathogenesis, particularly on responsiveness to different therapies; furthermore, there are implications to the treatment of AA in general.