Background: As opposed to large-cell or Burkitt's-type non-Hodgkin's lymphoma, acute lymphoblastic leukemia (ALL) is rarely observed in HIV-seropositive patients and is not a criteria of AIDS in the 1986 classification established by the C.D.C. Furthermore, the few cases of ALL reported so far were B-cell ALL, Burkitt's-type.
Design: We recently observed, with unexpected frequency, ALL cases in this setting: over a 5-year period, 5 of 25 HIV-positive patients referred to our center for hematological malignancies, had ALL. Three patients, who had previously been asymptomatic with regard to HIV infection, had typical Burkitt's-type ALL. Complete remission was achieved in all cases with high-dose lymphoma-type chemotherapy regimens, but 2 patients relapsed 3 and 27 months after diagnosis. Their clinical characteristics and outcome are discussed with reference to the cases reported thus far in the literature. One patient had common early pre-B ALL with the Philadelphia chromosome, and one had a T-cell ALL with an unusual CD7+, CD4-, CD8- phenotype. Prognosis was very poor in both cases.
Conclusion: The exact incidence and the therapeutic management of B-cell ALL in HIV-positive patients warrants further evaluation. In addition, we show that there may be a heterogeneity among ALL cases in this setting, with the first description of 2 non-Burkitt's ALL with atypical features in HIV-positive patients.