Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer-Cell Neoplasms.

Published

Journal Article (Review)

Hepatosplenic T-cell lymphoma is a rare, aggressive T-cell lymphoma, characterized by hepatosplenic sinusoidal infiltration of monotonous, medium-sized, nonactivated cytotoxic T cells, usually of γ/δ T-cell receptor type. Hepatosplenic T-cell lymphoma occurs more frequently in immunocompromised patients, especially in those receiving long-term immunosuppressive therapy. Patients usually manifest hepatosplenomegaly without lymphadenopathy. The bone marrow is also involved in two-thirds of cases and is often accompanied by circulating lymphoma cells, which, along with anemia and thrombocytopenia, may raise suspicion for acute leukemia. The differential diagnosis includes aggressive natural killer-cell leukemia, T-large granular lymphocytic leukemia, T-lymphoblastic leukemia, enteropathy-associated T-cell lymphoma type II, primary cutaneous γ/δ T-cell lymphoma, other peripheral T-cell lymphomas, myelodysplastic syndrome, and infectious mononucleosis. The diagnosis is usually established from the combination of clinical findings, histologic features, and immunophenotype, although cytogenetic/molecular studies are occasionally needed. Hepatosplenic T-cell lymphoma exhibits a dismal clinical course with a poor response to currently available therapies.

Full Text

Duke Authors

Cited Authors

  • Shi, Y; Wang, E

Published Date

  • September 2015

Published In

Volume / Issue

  • 139 / 9

Start / End Page

  • 1173 - 1180

PubMed ID

  • 26317456

Pubmed Central ID

  • 26317456

Electronic International Standard Serial Number (EISSN)

  • 1543-2165

Digital Object Identifier (DOI)

  • 10.5858/arpa.2014-0079-RS

Language

  • eng

Conference Location

  • United States