We studied the characteristics and temporal trends of AIDS- associated non-Hodgkin's lymphoma (AIDS-NHL) in individuals with hemophilia. Prospective data were collected on 33 HIV-positive hemophiliacs with AIDS-NHL enrolled in the Hemophilia Malignancy Study (HMS), of whom 21 had primary and 12 had secondary or subsequent AIDS-defining illnesses, and analyzed for frequency and temporal trends. As compared with primary AIDS- NHL, secondary AIDS-NHL occurred at an older mean age, 37 versus 29 years (p = 0.12); at a lower mean CD4 count, 46 versus 154 (p = 0.07); after a longer period of immunosuppression (CD4 < 200/microl), 41 versus 16 months (p = 0.03); and with shorter median survival, 2 versus 7 months (p = 0.09). The presence of EBV in tumor tissue was associated with shorter survival, 1 versus 7 months (p = 0.17). Between 1981 and 1988 and 1989 and 1994, the proportion of primary AIDS diagnoses that were AIDS-NHL changed minimally, 4.6 versus 6.1%, whereas there were significant decreases in Pneumocystis carinii pneumonia (PCP, p = 0.02) and wasting (p = 0.07), and an increase in Candida (p = 0.004). These findings confirm that an increasing proportion of AIDS-NHL in hemophiliacs are occurring as secondary or later AIDS diagnoses, and they are associated with prolonged duration of immunosuppression.

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A 52-year old woman presented with signs and symptoms of thoracic spinal cord compression caused by a spinal canal mass extending from the seventh to the ninth thoracic vertebra. Initial treatment consisted of high dose steroids and decompression laminectomy with subtotal resection of the abnormal mass. Sheets of immature myeloid cells which expressed the t(15;17)(q22;q21) of acute promyelocytic leukemia (APL) by fluorescence in situ hybridization (FISH) were present, consistent with promyelocytic sarcoma. Bone marrow examination showed no evidence of acute leukemia. She was then placed on the PETHEMA (Programa para el Tratamiento de Hematopatias Malignas, a Spanish cooperative chemotherapy group) regimen for APL for 15 months. There has been no evidence of local recurrence or of systemic APL at the 4.5-year mark, the longest follow-up of isolated promyelocytic sarcoma in the medical literature.