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T cell acute lymphoblastic leukemia (T-ALL) is a disease of poor prognosis, usually studied separately in adults or children. Controversial clinical or biological prognostic factors have been reported, and little information is available regarding the frequency and prognostic value of membrane markers identified on blast cells. We report an extensive investigation of the incidence and prognostic value of immunophenotypic, clinical, and laboratory data in T-ALL, performed as a multicenter study in 164 patients. CD7, CD5, and CD2 were the most frequently expressed T cell antigens, and CD2 and CD4 were more frequently observed in children than in adults. MHC class II, CD9, and CD10 were observed in 16, 22, and 21% of the patients, respectively. The male prevalence of T-ALL, and the more frequent presence of a tumoral syndrome in children were confirmed, but mediastinal enlargement and high leukocyte counts were observed in less than half the patients. A poor prognosis was associated with the expression of MHC class II in adults. The presence of a mediastinal mass appeared to be of good prognosis in adults, as well as a leukocyte count lower than 100 x 10(9)/l whatever the age of the patient.

Seventeen patients with adult T-cell leukemia (ATL) and 21 with tropical spastic paraparesis/human T-cell leukemia/lymphoma virus type I (HTLV-I)-associated myelopathy (TSP/HAM) were observed during a 3-yr survey (1986-1988) in some hospitals in Paris, France. Most of them were black, originating from high-HTLV-I-endemic areas (West Indies or Africa), but two cases of TSP/HAM occurred in French Caucasians. In one case, the patient acquired the virus from a transfusion during a cardiac transplantation. Most of the ATL cases were diagnosed as acute leukemia or lymphoma, with a proliferation of CD2+, CD3+, CD4+, CD8-, DR+, and CD25+ lymphoid cells. Only three cases were diagnosed as a smoldering ATL. All of the TSP/HAM cases exhibited a spastic paraparesis with a chronic and slow evolution and high HTLV-I antibody titers in serum and cerebrospinal fluid, with a high HTLV-I antibody index and specific HTLV-I immunoglobulin = oligoclonal bands. In TSP/HAM, a high percentage of DR-expressing cells (15 to 40%) was found, with a slightly elevated CD4/CD8 ratio. This was associated with the presence of 1 to 10% abnormally shaped nuclei in lymphoid cells and a polyclonal integration of HTLV-I proviruses in these peripheral blood mononuclear cells. On the contrary, a clonal integration was always found in the ATL malignant cells (leukemic, lymph node, and cutaneous infiltrate). Long-term interleukin 2-dependent T-cell lines (CD2+, CD3+, CD4+, and WT31+) with activated T-cell markers (CD25+ and DR+) producing HTLV-I were established from ATL and TSP/HAM peripheral blood mononuclear cells.