title: Nasal T-cell lymphoma associated with hemophagocytic syndrome. Immunohistochemical and genotypic studies.
abstract: A 53-year-old man with nasal T-cell lymphoma exhibited hemophagocytic syndrome as a terminal event. Immunohistochemical studies revealed that neoplastic cells were derived from T cells. Genotypic analysis of DNA samples that were obtained from the frozen tissue specimens demonstrated clonal rearrangements of the T-cell receptor beta-chain genes. No rearrangement was observed in the immunoglobulin heavy-chain gene. To our knowledge, only three cases of nasal T-cell lymphoma with hemophagocytic syndrome have been reported before the present case. All of these cases occurred in Oriental patients. The present report suggests the beneficial effect of high-dose glucocorticoid therapy on the prolongation of survival compared with that of the other three cases.
authors.affiliation: Third Department of Internal Medicine, Akita University School of Medicine, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Chubachi
authors.forename: H
authors.initials: H
authors.lastname: Imai
authors.forename: S
authors.initials: S
authors.lastname: Nishimura
authors.forename: M
authors.initials: M
authors.lastname: Saitoh
authors.forename: A B
authors.initials: AB
authors.lastname: Miura
mesh_terms: D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D005838:Genotype; D006644:Histiocytes / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000235:genetics / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity; D009669:Nose Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology; D013154:Spleen / Q000473:pathology