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Prepublished online as a Blood First Edition Paper on August 7, 2003; DOI 10.1182/blood-2003-05-1675. This Article Full Text (PDF) All Versions of this Article: 2003-05-1675v1 102/13/4261    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Belhadj, K. Articles by Gaulard, P. Search for Related Content PubMed PubMed Citation Articles by Belhadj, K. Articles by Gaulard, P. Social Bookmarking                   What's this? Submitted May 27, 2003 Accepted July 7, 2003 Hepatosplenic T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 cases Karim Belhadj, Felix Reyes, Jean-Pierre Farcet, Herve Tilly, Christian Bastard, Regis Angonin, Eric Deconinck, Frederic Charlotte, Veronique Leblond, Eric Labouyrie, Pierre Lederlin, Jean-Francois Emile, Beatrice Delmas-Marsalet, Bertrand Arnulf, Elie-Serge Zafrani, and Philippe Gaulard* Service d'Hematologie Clinique, CHU Henri Mondor, AP-HP, Creteil, France Departement de Pathologie, CHU Henri Mondor, AP-HP, Creteil, France; EA 2348, CHU Henri Mondor, AP-HP, Creteil, France Service d'Immunologie Biologique, CHU Henri Mondor, AP-HP, Creteil, France Service d'Hematologie Clinique, Centre Henri Becquerel, Rouen, France Laboratoire de Cytogenetique, Centre Henri Becquerel, Rouen, France Service d'Hematologie Clinique, CHU Jean Minjoz, Besancon, France Service d'Anatomie Pathologique, CHU Jean Minjoz, Besancon, France Service d'Hematologie Clinique, CHU Pitie-Salpetriere, AP-HP, Paris, France Service d'Anatomie Pathologique, CHU Pitie-Salpetriere AP-HP, Paris, France Service d'Hematologie Clinique, CHU de Brabois, Vandoeuvre les Nancy, France Service d'Anatomie Pathologique, CHU de Brabois, Vandoeuvre les Nancy, France Service d'Hematologie Clinique, CHU Paul Brousse, AP-HP, Villejuif, France Service d'Anatomie Pathologique, CHU Paul Brousse, AP-HP, Villejuif, France Service d'Hematologie Clinique, CHU Necker, AP-HP, Paris, France * Corresponding author; email: philippe.gaulard{at}hmn.ap-hop-paris.fr. We report on the characteristics of 21 cases of Hepatosplenic T-cell lymphoma (HSTCL), an entity recognized since 1994 by the REAL classification. Median age was 34 years. Patients presented with splenomegaly (n=21), hepatomegaly (n=15) and thrombocytopenia (n=20). Histopathologic findings were homogeneous with the presence of medium-sized lymphoma cells within the sinusoids of splenic red pulp, liver and bone marrow. Marrow involvement was usually mild but could be demonstrated by phenotyping in all cases. Cells were CD3+, CD5-, expressed the T-cell receptor and had a non-activated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most cases were CD4-/CD8- (16/18), CD56+ (15/18), expressed the V1epitope (Vd1+/Vd2-/Vd3-, 9/12) and were negative for EBV (18/20). An isochromosome 7q was found in nine of 13 documented cases. Eight patients had a previous history of kidney transplant, systemic lupus, Hodgkin disease or malaria. Prognosis was very poor with a median survival time of 16 months and all patients but two ultimately dying despite consolidative or salvage high-dose therapy. In conclusion, HSTCL is a disease with distinctive clinical, histopathologic and phenotypic characteristics. Bone marrow biopsy with combined phenotyping is sufficient for diagnosis and splenectomy is therefore unwarranted. Current treatment modalities appear to be ineffective in most cases. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. Ravandi, A. Aribi, S. O'Brien, S. Faderl, D. Jones, A. Ferrajoli, X. Huang, S. York, S. Pierce, W. Wierda, et al. Phase II Study of Alemtuzumab in Combination With Pentostatin in Patients With T-Cell Neoplasms J. Clin. Oncol., November 10, 2009; 27(32): 5425 - 5430. 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