title: Histiocytosis following T-acute lymphoblastic leukemia: a BFM study.
abstract: 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 ( approximately 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 microl(-1) vs 134,141 +/- 5,663 microl(-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 <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. The authors recommend a close follow-up for at least 3 years after diagnosis in younger T-ALL patients with high initial leukocyte count.
authors.affiliation: St Anna Children's Hospital, Vienna, Austria. m.trebo@utanet.at
authors.forename: Monika M
authors.initials: MM
authors.lastname: Trebo
authors.forename: Andishe
authors.initials: A
authors.lastname: Attarbaschi
authors.forename: Georg
authors.initials: G
authors.lastname: Mann
authors.forename: Milen
authors.initials: M
authors.lastname: Minkov
authors.forename: Rosi
authors.initials: R
authors.lastname: Kornmüller
authors.forename: Helmut
authors.initials: H
authors.lastname: Gadner
mesh_terms: D001854:Bone Marrow Cells / Q000473:pathology; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D005260:Female; D015614:Histiocytosis / Q000209:etiology*; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000473:pathology*; D008297:Male; D012189:Retrospective Studies