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Blood, 15 December 2003, Vol. 102, No. 13, pp. 4261-4269. Prepublished online as a Blood First Edition Paper on August 7, 2003; DOI 10.1182/blood-2003-05-1675. This Article Full Text 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 Citation Map 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. Related Collections Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Hepatosplenic T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients Karim Belhadj, Félix Reyes, Jean-Pierre Farcet, Hervé Tilly, Christian Bastard, Régis Angonin, Eric Deconinck, Frédéric Charlotte, Véronique Leblond, Eric Labouyrie, Pierre Lederlin, Jean-François Emile, Béatrice Delmas-Marsalet, Bertrand Arnulf, Elie-Serge Zafrani, and Philippe Gaulard From the Service d'Hématologie Clinique, the Service d'Immunologie Biologique and the Département de Pathologie and EA2348, Centre Hospitalier Universitaire (CHU) Henri Mondor, Assistance Publique-Hôpitaux de Paris (AP-HP), Créteil, France; Service d'Hématologie Clinique and Laboratoire de Cytogénétique, Centre Henri Becquerel, Rouen, France; Service d'Anatomie Pathologique and Service d'Hématologie Clinique, CHU de Besançon, Besançon, France; Service d'Anatomie Pathologique and Service d'Hématologie Clinique, CHU Pitié-Salpêtrière, Paris, France; Service d'Anatomie Pathologique and Service d'Hématologie Clinique, CHU de Nancy, Nancy, France; Service d'Anatomie Pathologie and Service d'Hématologie Clinique, CHU Paul Brousse, Villejuif, France; and Service d'Hématologie Clinique, CHU Necker, Paris, France. We report on the characteristics of 21 patients with hepatosplenic T-cell lymphoma (HSTCL), an entity recognized since 1994 in the Revised European American Lymphoma (REAL) classification. Median age was 34 years. Patients had splenomegaly (n = 21), hepatomegaly (n = 15), and thrombocytopenia (n = 20). Histopathologic findings were homogeneous and showed 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 patients. Cells were CD3+CD5-, expressed the T-cell receptor, and had a nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most patients were CD4-/CD8- (16 of 18); CD56+ (15 of 18), expressed the V1epitope (Vd1+/Vd2-/Vd3-) (9 of 12); and were negative for Epstein-Barr virus (EBV) (18 of 20). Isochromosome arm 7q was documented in 9 of 13 patients. Eight patients had previously undergone kidney transplantation or had a history of systemic lupus, Hodgkin disease, or malaria. Prognosis was poor; median survival time was 16 months, and all but 2 patients ultimately died 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 patients. (Blood. 2003;102:4261-4269) 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. 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