The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.
Between September and August 1991 818 previously untreated children and adolescents up to 18 years of age with acute lymphoblastic leukemia were entered into two modified BFM-protocols. Patients with B-ALL were excluded. From 1981 to 1987 524 patients were entered into the randomized multicenter study ALL VII/81 (modified ALL-BFM 81 protocol). Patients were divided into three risk groups standard (SR), medium (MR), high risk (HR) using the BFM risk factor. In a connecting study from 1988 to 1991 294 patients were registered on the stratified and randomized multicentric trial ALL VIII/87 (modified ALL-BFM 86 study). The main modification in study ALL VII/81 concerned the duration of treatment. Patients were randomized into two groups. The first group received as a late reinduction protocol III and then therapy was stopped. The second group received 6-MP and MTX for another six months. The other whole treatment strategy of ALL-BFM 81 was adopted. In protocol ALL VIII/87 the only modification was the reduction of the MTX dosage from 5 g/m2 to 1 g/m2 with an infusion time of 24 hours (leucovorin rescue 15 mg/m2 after 48 and 54 hours). The probability of the event-free-survival (EFS) for study ALL VII/81 was 59%. CNS events were significantly more frequent in standard risk patients with intermediate dose MTX (4 x 0.5 g/m2) compared with the irradiation group (18 Gy). The EFS for SR patients amounts to 61%, for MR patients to 59% and for HR patients to 36%. There was no significant difference of EFS for the two groups with different duration of therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
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