A Novel Translocation, t(2;5)(p23;q35), in Childhood Phagocytic Large T-Cell Lymphoma Mimicking Malignant Histiocytosis

We report a novel chromosome translocation—t(2;5)(p23;q35) or its variant, t(2;5;13)(p23;q35;q12)—in 3 patients with peripheral T-cell lymphoma. All 3 were female children who had peripheral lymphadenopathy without organomegaly and underwent complete remission with or without chemotherapy. Their tum...

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Bibliographic Details
Published in:Blood 1989-02, Vol.73 (3), p.806-813
Main Authors: Kaneko, Yasuhiko, Frizzera, Glauco, Edamura, Setsuko, Maseki, Nobuo, Sakurai, Masaharu, Komada, Yoshihiro, Sakurai, Minoru, Tanaka, Haruki, Sasaki, Masamichi, Suchi, Taizan, Kikuta, Atsushi, Wakasa, Haruki, Hojo, Hiroshi, Mizutani, Shuki
Format: Article
Language:English
Subjects:
Antigens, Differentiation - analysis
Antigens, Neoplasm - analysis
Biological and medical sciences
Child
Chromosome Aberrations - genetics
Chromosome Banding
Chromosome Disorders
Chromosomes, Human, Pair 2
Chromosomes, Human, Pair 5
Female
Gene Rearrangement, B-Lymphocyte, Heavy Chain
Gene Rearrangement, beta-Chain T-Cell Antigen Receptor
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
Hematologic and hematopoietic diseases
Humans
Ki-1 Antigen
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphoma, Non-Hodgkin - genetics
Lymphoma, Non-Hodgkin - pathology
Medical sciences
Phagocytosis
T-Lymphocytes
Translocation, Genetic
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Summary:We report a novel chromosome translocation—t(2;5)(p23;q35) or its variant, t(2;5;13)(p23;q35;q12)—in 3 patients with peripheral T-cell lymphoma. All 3 were female children who had peripheral lymphadenopathy without organomegaly and underwent complete remission with or without chemotherapy. Their tumors were characterized histologically by predominant large cells, at times showing phagocytosis, and immunologically by peripheral T-cell phenotype and expression of Ki-1 antigen and epi- thelial membrane antigen (EMA). Since the same translocation has been reported in the literature in 4 patients with malignant histiocytosis (MH), and our patients had histologic features suggestive of that disease, it is likely that many tumors previously interpreted as MH are actually phagocytic large T-cell lymphoma carrying this translocation.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V73.3.806.806