title: De novo CD3 negative hepatosplenic T-cell lymphoma: diagnostic challenges and pitfalls.
abstract: Hepatosplenic T-cell lymphoma is a rare and aggressive peripheral T-cell malignancy that is distinctively characterized by sinusoidal infiltration of mature medium-sized T lymphocytes in the spleen and liver. The neoplastic cells are classically surface CD3(+), CD2(+), CD5(-), CD4(-), and CD8(+/-) and manifest variable expression of markers associated with natural killer (NK) cells such as CD16 and CD56. In this article, we report the first case to date of a newly diagnosed de novo surface CD3(-) hepatosplenic T-cell lymphoma with circulating blastlike neoplastic cells expressing NK-cell-associated markers. The lack of surface CD3 expression, together with the expression of NK-cell-associated markers and the leukemic presentation, leads to significant diagnostic challenges in differentiating this CD3(-) hepatosplenic T-cell lymphoma from NK-cell neoplasms, in particular aggressive NK-cell leukemia. The related literature is reviewed, and the approaches for adequate diagnosis of this novel situation are described.
authors: From the Department of Pathology and Laboratory Medicine, Orlando Health (Dr Harn Kapur), Department of Hematology/Oncology, Florida Hospital Cancer Institute (Drs Khaled and Solh), and Department of Pathology, Florida Hospital, University of Central Florida School of Medicine (Drs Ward and Chang), Orlando.
authors: Lucy Harn
authors: LH
authors: Kapur
authors: Yasser
authors: Y
authors: Khaled
authors: Melhem
authors: M
authors: Solh
authors: David
authors: D
authors: Ward
authors: Chung-Che
authors: CC
authors: Chang
mesh_terms: D000293:Adolescent; D017252:CD3 Complex / Q000378:metabolism*; D003937:Diagnosis, Differential; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis / Q000276:immunology; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000276:immunology*; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000276:immunology*; D008297:Male; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000276:immunology*
chemical_list: D017252:CD3 Complex