Distinguishing Between Hepatosplenic T-cell Lymphoma and gamma delta T-cell Large Granular Lymphocytic Leukemia A Clinicopathologic, Immunophenotypic, and Molecular Analysis

作者：Yabe Mariko; Medeiros L Jeffrey; Wang Sa A; Tang Guilin; Bueso Ramos Carlos E; Jorgensen Jeffrey L; Bhagat Govind; Chen Weina; Li Shaoying; Young Ken H; Miranda Roberto N^*

来源：American Journal of Surgical Pathology, 2017, 41(1): 82-93.

DOI：10.1097/PAS.0000000000000743

• hepatosplenic T-cell lymphoma
• gamma delta T-cell large granular lymphocytic leukemia
• T-cell lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare, aggressive T-cell lymphoma that can be challenging to diagnose. In particular, distinguishing HSTCL from T-cell large granular lymphocytic (T-LGL) leukemia of gamma delta T-cell receptor (TCR) type is difficult without examination of a splenectomy specimen. In this study, we systematically assessed a series of HSTCL cases for findings reported in the literature as supporting or not supporting the diagnosis of HSTCL. We also compared HSTCL with a group of cases of T-LGL of gamma delta d TCR type. Criteria assessed in this study included: B-symptoms, massive splenomegaly, lymphadenopathy, extranodal involvement, peripheral lymphocytosis, lymphoma cells that expand bone marrow sinuses, lymphocyte azurophilic granules, immunophenotype, evidence of infection by Epstein-Barr virus, human immunodeficiency virus, or human T-cell leukemia virus type 1, isochromosome 7q, trisomy 8, and TCR gene rearrangement status. On the basis of the data of this study, we conclude that massive splenomegaly, bone marrow sinusoidal expansion by lymphoma cells, and lymphocytes devoid of azurophilic granules were significantly more common in HSTCL patients than in gamma delta T-LGL patients (P<0.0001), and these features help support the diagnosis of HSTCL.

• 出版日期2017-1