title: [Definition of malignant histiocytosis and large cell anaplastic (Ki-1) lymphoma in children].
abstract: Cells belonging to the mononuclear phagocytic system may give rise to a wide variety of proliferative disorders. These neoplastic or reactive diseases of monocytes, histiocytes and macrophages are still poorly understood, both from a biological, clinical and pathological point of view. In 1984 a new type of non-Hodgkin's lymphomas has been described, which recently was termed large cell anaplastic lymphoma (LCAL). Since both "true" malignant histiocytosis (MH) and LCAL display some clinico-pathological similarities, we reevaluated eleven children with the primary diagnosis of MH, who entered the multicentric therapy study DAL-HX 83. On the basis of the typical morphology LCAL and not MH has been diagnosed in all cases. The Ki-1 (CD 30) positive tumor cells of LCAL can display different phenotypes, either T-lymphoid, B-lymphoid, histiocytic or neither of them (O-phenotype). Therefore we investigated the lineage specificity of tumor cells in 15 paediatric patients with LCAL. Tumor cells of histiocytic origin could be demonstrated in only one of them. In 7 patients the neoplastic cells were of T cell and in the remaining 7 cases of non T/non B cell (O) phenotypes, confirming the extreme rarity of MH in children. It is our opinion that MH and LCAL seem to be different diseases not only nosologically but also clinico-pathologically. In the multicentric therapy studies NHL-BFM 81 and NHL-BFM 83 the frequency of LCAL in childhood is compared to all other subentities at 3%.
authors.affiliation: Kinderklinik der Medizinischen Hochschule Hannover.
authors.forename: P
authors.initials: P
authors.lastname: Bucsky
authors.forename: A C
authors.initials: AC
authors.lastname: Feller
authors.forename: J D
authors.initials: JD
authors.lastname: Beck
authors.forename: H
authors.initials: H
authors.lastname: Gadner
authors.forename: A
authors.initials: A
authors.lastname: Heitger
authors.forename: W D
authors.initials: WD
authors.lastname: Ludwig
authors.forename: A
authors.initials: A
authors.lastname: Reiter
authors.forename: H
authors.initials: H
authors.lastname: Riehm
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal; D000943:Antigens, Differentiation / Q000032:analysis*; D000951:Antigens, Neoplasm / Q000032:analysis*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D018219:Histiocytoma, Benign Fibrous / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D017730:Ki-1 Antigen; D008198:Lymph Nodes / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal; D000943:Antigens, Differentiation; D000951:Antigens, Neoplasm; D017730:Ki-1 Antigen
vernacular_title: Zur Frage der Definition der malignen Histiozytose und des grosszelligen anaplastischen (Ki-1) Lymphoms im Kindesalter.