Hepatosplenic T-cell lymphoma: A clinicopathologic review with an emphasis on diagnostic differentiation from other T-cell/natural killer-cell neoplasms

Yang Shi, Endi Wang

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Hepatosplenic T-cell lymphoma is a rare, aggressive Tcell 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.

Original languageEnglish (US)
Pages (from-to)1173-1180
Number of pages8
JournalArchives of Pathology and Laboratory Medicine
Volume139
Issue number9
DOIs
StatePublished - Sep 1 2015
Externally publishedYes

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T-Cell Lymphoma
Large Granular Lymphocytic Leukemia
Natural Killer Cells
T-Lymphocytes
Lymphoma
Enteropathy-Associated T-Cell Lymphoma
Peripheral T-Cell Lymphoma
Cutaneous T-Cell Lymphoma
Neoplasms
Infectious Mononucleosis
Myelodysplastic Syndromes
Immunocompromised Host
Immunosuppressive Agents
T-Cell Antigen Receptor
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Cytogenetics
Thrombocytopenia
Anemia
Leukemia
Differential Diagnosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology
  • Medicine(all)

Cite this

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abstract = "Hepatosplenic T-cell lymphoma is a rare, aggressive Tcell 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.",
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