EBV-negative Aggressive NK-cell Leukemia/Lymphoma: Clinical, Pathologic, and Genetic Features.
Fiche publication
Date publication
janvier 2017
Journal
The American journal of surgical pathology
Auteurs
Membres identifiés du Cancéropôle Est :
Dr NICOLAE Alina
Tous les auteurs :
Nicolae A, Ganapathi KA, Pham TH, Xi L, Torres-Cabala CA, Nanaji NM, Zha HD, Fan Z, Irwin S, Pittaluga S, Raffeld M, Jaffe ES
Lien Pubmed
Résumé
Aggressive natural killer cell leukemia (ANKL) is a systemic NK-cell neoplasm, almost always associated with Epstein-Barr virus (EBV). Rare cases of EBV-negative ANKL have been described, and some reports suggested more indolent behavior. We report the clinicopathologic, immunophenotypic, and molecular characteristics of 7 EBV-negative ANKL. All patients were adults, with a median age of 63 years (range 22 to 83 y) and an M:F ratio of 2.5:1. Five patients were White, 1 Black, and 1 Asian. All patients presented acutely, with fever (6/7), cytopenias (6/7), and splenomegaly (4/7). Four patients had lymphadenopathy, 4 had extranodal disease. Bone marrow involvement was present in 5, with hemophagocytosis in 3. Peripheral blood was involved in 5 with the neoplastic cells containing prominent azurophilic granules. By immunohistochemistry and/or flow cytometry, the tumor cells lacked surface CD3 and were positive for CD56 (7/7), CD2 (5/5), CD8 (3/7), CD30 (4/5), and granzyme-B (6/6). They were negative for CD4, CD5, βF1, TCRγ, LMP1, and EBV-encoded RNA. Polymerase chain reaction for TCRG clonality was polyclonal. Mutational analysis revealed missense mutations in the STAT3 gene in both cases studied. Median survival was 8 weeks from the onset of disease. One patient received allogeneic bone marrow transplant and is alive with no disease (follow-up 15 mo). EBV-negative ANKL exists but is rare. It tends to occur in older patients and is indistinguishable clinically and pathologically from EBV-positive ANKL, with a similar fulminant clinical course. The high prevalence of Asian patients seen with EBV-positive disease seems less evident with EBV-negative cases.
Mots clés
Aged, Aged, 80 and over, DNA Mutational Analysis, Epstein-Barr Virus Infections, Female, Flow Cytometry, Humans, Immunohistochemistry, Immunophenotyping, In Situ Hybridization, Leukemia, Large Granular Lymphocytic, genetics, Male, Middle Aged, Polymerase Chain Reaction, Young Adult
Référence
Am J Surg Pathol. 2017 01;41(1):67-74