Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment

Falchook, G. S.; Vega, Francisco; Dang, Nam H.; Samaniego, Felipe; Rodriguez, Maria Alma; Champlin, Richard E.; Hosing, Chitra; Verstovšek, Srđan; Pro, Barbara

doi:10.1093/annonc/mdn751

Cited by 135 publications

(125 citation statements)

References 27 publications

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“…The median survival in that series was 16 months. Occasional patients have been reported to have prolonged survival after treatment with alemtuzumab and cladribine [94], pentostatin [95], and allogeneic hematopoietic stem cell transplantation [96,97]. A recent report described long-term disease-free survival in seven of 14 patients who received high-dose therapy and autologous stem cell transplant as part of their initial therapy [98].…”

Section: Hepatosplenic T-cell Lymphomamentioning

confidence: 99%

The aggressive peripheral T‐cell lymphomas: 2015

Armitage

2015

American J Hematol

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Background: T‐cell lymphomas make up approximately 10%–15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia.Diagnosis: The diagnosis of aggressive peripheral T‐cell lymphoma (PTCL) is usually made using the World Health Organization classification. The ability of hematopathologists to reproducibly diagnosis aggressive PTCL is lower than that for aggressive B‐cell lymphomas, with a range of 72%–97% for the aggressive PTCLs.Risk Stratification: Patients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, positron emission tomography scans seem to be useful as they are in aggressive B‐cell lymphomas. The most commonly used prognostic index is the International Prognostic Index. The specific subtype of aggressive PTCL is an important risk factor, with the best survival seen in anaplastic large‐cell lymphoma—particularly young patients with the anaplastic lymphoma kinase positive subtype.Risk‐Adapted Therapy: Anaplastic large‐cell lymphoma is the only subgroup to have a good response to a CHOP‐like regimen. Angioimmunoblastic T‐cell lymphoma has a prolonged disease‐free survival in only ∼20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL‐not otherwise specified is not one disease. Anthracycline‐containing regimens have disappointing results, and a new approach is needed. Natural killer/T‐cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy‐containing regimens. Enteropathy‐associated PTCL and hepatosplenic PTCL are rare disorders with a generally poor response to therapy, although selected patients with enteropathy‐associated PTCL seem to benefit from intensive therapy. Am. J. Hematol. 90:666–673, 2015. © 2015 Wiley Periodicals, Inc.

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Section: Hepatosplenic T-cell Lymphomamentioning

confidence: 99%

The aggressive peripheral T‐cell lymphomas: 2015

Armitage

2015

American J Hematol

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“…6 HSTL is characterized by a monotonous neoplastic infiltrate consisting of medium-sized lymphocytes with a moderate amount of cytoplasm and indistinct cellular borders. [8][9][10][11][12][13][14] The nucleus is oval, often slightly indented or clearly folded, with chromatin less condensed than that of small lymphocytes. The nucleoli, when present, are small, clearly visible.…”

Section: Discussionmentioning

confidence: 99%

γδ T-cell lymphoma: a rare entity mimicking hemophagocytic syndrome-report of two cases

et al. 2017

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γδ T-cell lymphoma is a rare extranodal and systemic neoplasm which accounts for less than 1% of all NHL having peak incidence in adolescents and young adults with a male predominance. Study report two cases, 29 and 25 years male presented with fever, abdominal pain and pancytopenia; one had hepatosplenomegaly while other had marked splenomegaly respectively and both showing feature of hemophagocytosis. Both underwent splenectomy. On gross examination, the spleen was markedly enlarged and shows a deep brown cut-surface. Histologically the normal structure of the spleen is totally effaced by a monotonous lymphoid population consisting of medium-large sized lymphoid cells with vesicular chromatin and small eosinophilic nucleolus with tumor cells showing moderate amount of eosinophilic cytoplasm. Liver biopsy showed regenerative changes in parenchyma and sinusoidal involvement by tumour cells. Peripheral smear revealed atypical lymphocytes with pancytopenia. Bone marrow aspiration showed moderate neoplastic infiltrate by lymphoid cells. Immunophenotypic study showed tumour cells expressing CD3, CD56, CD 2 (dim), TIA-1, TCR-gamma. The tumour cells were immunonegative for CD20, CD5, CD4, CD8, granzyme B, CD7, CD10 and CD 23. Immunophenotypic study is must for diagnosis of γδ T-cell lymphoma which can mimic hemophagocytic syndrome.

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“…[1,2] Initially, the term 'gamma-delta hepatosplenic T-cell lymphoma was proposed as a provisional entity in the Revised European-American Lymphoma (REAL) classification. However, identification of patients who demonstrated an alpha-beta phenotype with the clinicopathologic features of HSTCL, the term 'hepatosplenic T-cell lymphoma' was adopted for use in the current World Health Organization classification [3][4][5][6][7][8].…”

Section: Discussionmentioning

confidence: 99%

Hepatosplenic T‐Cell Lymphoma

Gatter

Delsol

Warnke

2011

The Diagnosis of Lymphoproliferative Diseases

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We report an uncommon case of 38-year-old male patient with Hepatosplenic T-Cell lymphoma (HSTCL) which is a rare aggressive form of Peripheral T-Cell lymphoma that is characterized by primary extranodal disease with malignant T-cell proliferation in the liver, spleen, and bone marrow. Our patient presented with progressive painless jaundice, weight loss and massive hepatosplenomegaly. The diagnosis was challenging as he required an extensive investigations that ultimately showed the characteristic clinical, histopathologic, and cytogenetic features of hepatosplenic T-cell lymphoma.Keywords Hepatosplenic Á Lymphoma Á Hepatosplenomegaly Hepatosplenic T-Cell Lymphoma (HSTCL) is an uncommon neoplasm that comprises 5% of peripheral T-cell lymphomas. The majority of cases express the cd T-cell receptor, however, recently, a small number of cases have been reported to express the ab T-cell receptor. Typical clinical features of this lymphoma include a predominance of young male patients, an aggressive clinical course, massive hepatosplenomegaly with sinusoidal infiltration of the liver, spleen, and bone marrow, minimal lymphadenopathy, expression or rearrangement of the cd T-cell receptor, and frequent presence of an isochromosome 7q and trisomy 8. This case highlights the importance of considering diagnosis of HSTCL in patients presenting with jaundice and massive hepatosplenomegaly. CaseA 38-year-old Filipino male presented with a history of lethargy, painless jaundice, dark urine, weight loss (5 kg), and progressive abdominal distension over 3-month-period. He denied history of blood transfusion, significant alcohol consumption and high risk behaviors. He had no history of drug ingestion including acetaminophen and use of herbal remedies. On physical examination; he looked well but deeply jaundiced. His vital signs were stable. There were no lymphadenopathy or peripheral stigmata of chronic liver disease. His abdomen was distended with massive hepatosplenomegaly but without clinically detectable ascites.His initial blood work showed: hemoglobin of 122 g/l, WBCs count of 3.8 9 10 9 /l (73% neutrophils, 16% lymphocytes, 3% monocytes, 1% eosinophils), platelets of 114 9 10 9 /l and his peripheral blood film showed mild pancytopenia with mild atypical reactive lymphocytes. The rest of investigations were as follows: albumin 39 g/l, bilirubin (total 63 lmol/l, direct 48 lmol/l), ALT 355 IU/l, alkaline phosphatase 719 IU/l, creatinine 65 lmol/l, prothrombin time 13.5 s. Serology for common hepatotropic viruses including HAV IgG, HBsAg, anti-HCV and quantitative EBV were negative. Screening tests for human immunodeficiency virus, malaria, schistosomiasis, leishmaniasis, tuberculosis, and common metabolic and autoimmune disorders were also negative. CT scan of the abdomen showed massive hepatosplenomegaly with minimal ascites and scattered small mesenteric lymph nodes.

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Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment

Abstract: A better understanding of the pathophysiology of HSTCL and new therapeutic strategies are needed.

Cited by 135 publications

References 27 publications

The aggressive peripheral T‐cell lymphomas: 2015

The aggressive peripheral T‐cell lymphomas: 2015

γδ T-cell lymphoma: a rare entity mimicking hemophagocytic syndrome-report of two cases

Hepatosplenic T‐Cell Lymphoma

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