title: Hepatosplenic T-cell lymphoma: A case series.
abstract: Hepatosplenic T-cell lymphoma (HSTCL) is a rare type of Non-Hodgkin Lymphoma (NHL), grouped under the mature or peripheral T-cell lymphomas. It is characterised by extranodal infiltration and proliferation of malignant T-cells within the sinusoids of the liver, sinuses and red pulp of the spleen, and the bone marrow. The tumour cells express CD2 and CD3, but are CD4, CD5 and CD8 negative and express a clonally restricted gamma-delta (or less commonly alpha-beta) T-cell receptor. The disease has an aggressive clinical course associated with a poor prognosis. We highlight and report three patients from South Africa with HSTCL, all of whom had hepatosplenomegaly and cytopaenias, and despite being HIV seronegative and immunocompetent, had a poor outcome, with a mean survival of 7.5 months in the two evaluable patients. This rare entity has not previously been reported from South Africa and as yet needs to be adequately characterised in a population where lymphoma is the most common haematological malignancy in adults, and where approximately two thirds of the adult lymphoma population are HIV seropositive.
authors.affiliation: Division of Clinical Haematology, Department of Medicine, Chris Hani Baragwanath Academic Hospital and Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Philippa
authors.initials: P
authors.lastname: Ashmore
authors.affiliation: Division of Clinical Haematology, Department of Medicine, Chris Hani Baragwanath Academic Hospital and Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. Electronic address: moosa.patel@wits.ac.za.
authors.forename: Moosa
authors.initials: M
authors.lastname: Patel
authors.affiliation: Department of Molecular Medicine and Haematology, NHLS (National Health Laboratory Services), Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Jenifer
authors.initials: J
authors.lastname: Vaughan
authors.affiliation: Department of Molecular Medicine and Haematology, NHLS (National Health Laboratory Services), Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Tracey
authors.initials: T
authors.lastname: Wiggill
authors.affiliation: Department of Molecular Medicine and Haematology, NHLS (National Health Laboratory Services), Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Pascale
authors.initials: P
authors.lastname: Willem
authors.affiliation: Department of Anatomical Pathology, NHLS (National Health Laboratory Services), Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Eunice
authors.initials: E
authors.lastname: van den Berg
authors.affiliation: Division of Clinical Haematology, Department of Medicine, Chris Hani Baragwanath Academic Hospital and Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Vinitha
authors.initials: V
authors.lastname: Philip
authors.affiliation: Division of Clinical Haematology, Department of Medicine, Chris Hani Baragwanath Academic Hospital and Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Atul
authors.initials: A
authors.lastname: Lakha
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008113:Liver Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology*; D008297:Male; D011379:Prognosis; D013019:South Africa / Q000453:epidemiology; D013154:Spleen / Q000473:pathology; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology*; D055815:Young Adult
chemical_list: D015703:Antigens, CD
keywords: Aggressive course; Cytopaenias; Hepatosplenic T-cell lymphoma; Hepatosplenomegaly; Poor prognosis; South Africa