title: A clinicopathological study of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome with special reference to nasal and nasal-type NK/T-cell lymphoma.
abstract: We describe the clinicopathological features of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome (T/NK-LAHS). These patients were categorized into 2 groups according to the onset of hemophagocytic syndrome (HPS). Group 1 developed HPS during the clinical course, typically at the terminal phase of the disease. This group consisted of 7 patients with extranodal lymphoma arising in the nasal cavity, paranasal cavity, tonsils, or skin at presentation. In 5 of these patients, the preferred diagnosis was nasal and nasal-type NK/T-cell lymphoma, whereas the disease diagnoses in the remaining 2 patients were peripheral T-cell lymphoma of unspecified type and angioimmunoblastic T-cell lymphoma, respectively. Group 2 consisted of 13 patients whose disease corresponded to so-called malignant histiocytosis-like lymphoma, which is characterized by HPS at the initial presentation and the infiltration of the liver, spleen, and/or bone marrow without tumor formation. Nine of these 13 cases were found to have common histopathological features: CD56+, Epstein-Barr virus positivity, cytotoxic molecules, and nasal-type NK/T-cell lymphoma. The very poor prognosis of T/NK-LAHS may be partly explained by the finding that nasal and nasal-type NK/T-cell lymphoma, which is resistant to standard chemotherapy, made up the highest percentage (70%) of the cases.
authors.affiliation: Third Department of Internal Medicine, Akita University School of Medicine, Japan. naoto@doc.med.akita-u.ac.jp
authors.forename: N
authors.initials: N
authors.lastname: Takahashi
authors.forename: I
authors.initials: I
authors.lastname: Miura
authors.forename: A
authors.initials: A
authors.lastname: Chubachi
authors.forename: A B
authors.initials: AB
authors.lastname: Miura
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019002:CD56 Antigen / Q000032:analysis; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology / Q000473:pathology* / Q000821:virology; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000473:pathology* / Q000821:virology; D016399:Lymphoma, T-Cell / Q000145:classification / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000401:mortality / Q000473:pathology* / Q000821:virology; D012367:RNA, Viral / Q000097:blood; D012189:Retrospective Studies; D015996:Survival Rate
chemical_list: D019002:CD56 Antigen; D012367:RNA, Viral