Vega F, Medeiros LJ, Gaulard P. Hepatosplenic and other gamma delta T-cell lymphomas
ABSTRACT The 2005 Society for Hematopathology/European Association for Haematopathology Workshop session 11 was dedicated to hepatosplenic T-cell lymphoma (HSTCL). HSTCL is a rare aggressive type of extranodal lymphoma characterized by hepatosplenomegaly, bone marrow involvement, and peripheral blood cytopenias. HSTCL exhibits a distinctive pattern of infiltration; tumor cells preferentially infiltrate the sinusoids of the splenic red pulp, liver, and bone marrow. The tumor cells have a nonactivated cytotoxic T-cell immunophenotype and frequently carry a recurrent cytogenetic abnormality, isochromosome 7q. Most cases express the gammadelta T-cell receptor, but cases can have an alphabeta phenotype and are considered to be a variant of the disease. Although HSTCL is the prototype peripheral T-cell lymphoma expressing the gammadelta T-cell receptor, non-HSTCL proliferations of gammadelta T cells can involve other extranodal sites, mainly skin and mucosa. These gammadelta T-cell lymphomas display marked heterogeneity in clinical and histologic features. In contrast with HSTCL, non-HSTCL gammadelta T-cell lymphomas frequently have an activated cytotoxic phenotype and most likely are not a single disease entity.
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- "Until recently, expression of the γδ TCR in lymphoma samples could only be assessed by flow cytometry or by immunohistochemistry on frozen sections. As a consequence, in routinely processed fixed material the γδ phenotype was extrapolated from the negativity for the αβ TCR (recognized by the anti-βF1 antibody) . "
Article: New biomarkers in T-cell lymphomas[Show abstract] [Hide abstract]
ABSTRACT: Peripheral T-cell lymphomas (PTCLs) are heterogeneous and uncommon malignancies characterized by an aggressive clinical course and a mostly poor outcome with current treatment strategies. The recent genome-wide molecular characterization of several entities has provided novel insights into their pathobiology and led to the identification of new biomarkers with diagnostic, prognostic or therapeutic implications for PTCL patients. Cell lineage and differentiation antigens (markers of γδ or NK lineage, of cytotoxicity, of follicular helper T cells) reflect the tumour's biological behaviour, and their detection in tissue samples may refine the diagnostic and prognostic stratification of the patients. Previously unrecognized gene rearrangements are being discovered (ITK-SYK translocation, IRF4/MUM1 and DUSP22 rearrangements), and may serve as diagnostic genetic markers. Deregulated molecules within oncogenic pathways (NF-κB, Syk, PDGFRα) and immunoreactive cell-surface antigens (CD30, CD52) have been brought to the fore as potential targets for guiding the development of novel therapies.Best practice & research. Clinical haematology 03/2012; 25(1):13-28. DOI:10.1016/j.beha.2012.01.004 · 2.12 Impact Factor
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- "Malignant neoplasms arising from γδ T-lymphocytes are very rare. Among those, the HSTCL represents <5% of peripheral T/ NK-lymphomas, and is less common than αβ peripheral T cell lymphoma.3 Only 150 cases have been described in literature since the initial description by Farcet and Gaulard in 1990. "
ABSTRACT: Hepatosplenic γδ T-cell lymphoma (HSTCL) is a very rare peripheral T-cell lymphoma characterized by extranodal infiltration of mature malignant post-thymic T-lymphocytes into sinusoids of the liver and spleen without lymphadenopathy and significant cytopenias. The aetiology of the disease is unknown. We describe the case of a female patient in whom HSTCL developed after delivery and who was previously without disease. Flow cytometry and liver puncture are essential for diagnosing HSTCL, especially in patients with unexplained pancytopenia and hepatosplenomegaly. Since phenotypic results can easily be misinterpreted as non-malignant, the examiner should have enough experience to recognize clonal changes of T-lymphocytes. Namely, in contrast to B-lymphocytes, T-lymphocytes do not have an efficient indicator of clonality and are recognized by flow cytometry based only on aberrant expression of commonly present antigens of T-cell and NK-cell subsets. At present, there is no known cure for HSTCL with a maximum survival up to 2 years.Hematology Reports 01/2012; 4(1):e4. DOI:10.4081/hr.2012.e4
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- "Although there are several reports of cases of hepatic or splenic primary lymphomas as separate entities, primary hepatosplenic lymphomas represent a rare entity, and the majority of cases reported have been of T-cell origin, especially the γ/δ subtype , although the presentation of primary hepatosplenic DLBCL is limited to the four cases analyzed in this paper (tables 1 and 2) [4, 5, 6], including our own case. Considering the small number of patients, it is not possible to establish definitive conclusions. "
ABSTRACT: Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.Case Reports in Gastroenterology 03/2008; 2(1):109-15. DOI:10.1159/000120757