Histiocytosis following T-acute lymphoblastic leukemia: A BFM study

The coincidence of T-cell acute lymphoblastic leukemia (T-ALL) and histiocytic disorders, including hemophagocytic lymphohistiocytosis (T-ALL HLH) and Langerhans cell histiocytosis (T-ALL LCH), is very seldom and is usually associated with a dismal prognosis. Retrospective statistical analysis of al...

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Veröffentlicht in:Leukemia & lymphoma 2005-12, Vol.46 (12), p.1735-1741
Hauptverfasser: Trebo, Monika M., Attarbaschi, Andishe, Mann, Georg, Minkov, Milen, Kornmüller, Rosi, Gadner, Helmut
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container_end_page 1741
container_issue 12
container_start_page 1735
container_title Leukemia & lymphoma
container_volume 46
creator Trebo, Monika M.
Attarbaschi, Andishe
Mann, Georg
Minkov, Milen
Kornmüller, Rosi
Gadner, Helmut
description The coincidence of T-cell acute lymphoblastic leukemia (T-ALL) and histiocytic disorders, including hemophagocytic lymphohistiocytosis (T-ALL HLH) and Langerhans cell histiocytosis (T-ALL LCH), is very seldom and is usually associated with a dismal prognosis. Retrospective statistical analysis of all T-ALL patients, who have been registered in the BFM-ALL trials from 1981 - 2001 and who have subsequently developed a LCH HLH, in order to identify any common risk factors pre-disposing to the synchronous occurrence of both disorders. Six out of 971 T-ALL patients had either HLH or LCH ( 0.03% of treated T-ALL year). The mean age at diagnosis of T-ALL HLH LCH was significantly lower than in the remaining T-ALL group (4.05 ± 0.59 vs 8.82 ± 0.14 years; p = 0.000). The mean initial leukocyte count was higher than in the non-HLH LCH group (270 700 ± 60 677 μl−1 vs 134 141 ± 5663 μl−1; p = 0.074). No hemophagocytosis was seen in the initial bone marrow (BM) smears. Five of 6 patients obtained a good prednisone response (GPR) at day 8 in peripheral blood with
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Retrospective statistical analysis of all T-ALL patients, who have been registered in the BFM-ALL trials from 1981 - 2001 and who have subsequently developed a LCH HLH, in order to identify any common risk factors pre-disposing to the synchronous occurrence of both disorders. Six out of 971 T-ALL patients had either HLH or LCH ( 0.03% of treated T-ALL year). The mean age at diagnosis of T-ALL HLH LCH was significantly lower than in the remaining T-ALL group (4.05 ± 0.59 vs 8.82 ± 0.14 years; p = 0.000). The mean initial leukocyte count was higher than in the non-HLH LCH group (270 700 ± 60 677 μl−1 vs 134 141 ± 5663 μl−1; p = 0.074). No hemophagocytosis was seen in the initial bone marrow (BM) smears. Five of 6 patients obtained a good prednisone response (GPR) at day 8 in peripheral blood with &lt;5% blasts at day 15 in BM and all cases were in complete remission (CR) at day 33. The mean time until development of the histiocytosis was 17.95 months (range 2.5 - 33 months). Four patients developed a HLH and 2 a LCH. All patients with HLH showed a multi-organ involvement, while the LCH patients had only local disease. Only the LCH patients survived, while all patients with HLH died. 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Retrospective statistical analysis of all T-ALL patients, who have been registered in the BFM-ALL trials from 1981 - 2001 and who have subsequently developed a LCH HLH, in order to identify any common risk factors pre-disposing to the synchronous occurrence of both disorders. Six out of 971 T-ALL patients had either HLH or LCH ( 0.03% of treated T-ALL year). The mean age at diagnosis of T-ALL HLH LCH was significantly lower than in the remaining T-ALL group (4.05 ± 0.59 vs 8.82 ± 0.14 years; p = 0.000). The mean initial leukocyte count was higher than in the non-HLH LCH group (270 700 ± 60 677 μl−1 vs 134 141 ± 5663 μl−1; p = 0.074). No hemophagocytosis was seen in the initial bone marrow (BM) smears. Five of 6 patients obtained a good prednisone response (GPR) at day 8 in peripheral blood with &lt;5% blasts at day 15 in BM and all cases were in complete remission (CR) at day 33. The mean time until development of the histiocytosis was 17.95 months (range 2.5 - 33 months). 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source MEDLINE; Taylor & Francis:Master (3349 titles); Taylor & Francis Medical Library - CRKN
subjects Acute lymphoblastic leukemia
bone marrow biopsy
Bone Marrow Cells - pathology
chemotherapy
Child, Preschool
Clinical Trials as Topic
Female
hemophagocytic lymphohistiocytosis
Histiocytosis - etiology
Histiocytosis, Langerhans-Cell - pathology
Humans
Langerhans cell histiocytosis
Leukemia-Lymphoma, Adult T-Cell - pathology
Male
Retrospective Studies
title Histiocytosis following T-acute lymphoblastic leukemia: A BFM study
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