mercaptopurine and Immunoblastic-Lymphadenopathy

A five-month-old girl developed high fever, erythema, hepatosplenomegaly and generalized lymphadenopathy. Laboratory examinations revealed elevated peripheral leukocyte counts, C-reactive protein, lactate dehydrogenase and serum ferritin level. Pathologic examination of the lymph nodes revealed immunoblastic lymphadenopathy (IBL) on the basis of the complete effacement of the normal architecture, replacement by a diffuse infiltrate composed of immunoblasts, plasmacytoid cells and small lymphocytes, and an abortive proliferation of blood vessels. B-cells and T-cells were nearly equally mixed throughout the lymph nodes. No rearrangements of the B-cell immunoglobulin and T-cell receptor genes were detected. The patient was initially treated with alpha-interferon with dramatic efficacy. After relapse, however, the disease was well controlled with cyclosporin A (CyA) and subsequently with combination regimens of CyA, deoxyspagarin and azathioprine with fair success. An alternating regimen of 6-mercaptopurine, cyclophosphamide and methotrexate was then instituted which continued the complete remission for 12 months. The effects of immunosuppressants in the treatment of IBL merit investigation.

Topics: Azathioprine; Cyclophosphamide; Cyclosporine; Drug Therapy, Combination; Female; Humans; Immunoblastic Lymphadenopathy; Immunosuppressive Agents; Infant; Interferon-alpha; Lymph Nodes; Mercaptopurine; Methotrexate; Recurrence; Remission Induction