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Hepatosplenic T-cell lymphoma

MedGen UID:
272711
•Concept ID:
C1333984
•
Neoplastic Process
Synonyms: Hepatosplenic Gamma/Delta T-Cell Lymphoma; Hepatosplenic gamma/Delta T-cell lymphoma; Hepatosplenic T-Cell Lymphoma; hepatosplenic T-cell lymphoma; HSTCL
SNOMED CT: Hepatosplenic T-cell lymphoma (445406001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019474
Orphanet: ORPHA86882

Definition

A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly, pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHepatosplenic T-cell lymphoma
Follow this link to review classifications for Hepatosplenic T-cell lymphoma in Orphanet.

Professional guidelines

PubMed

Few and far between: clinical management of rare extranodal subtypes of mature T-cell and NK-cell lymphomas.
Stuver R, Epstein-Peterson ZD, Horwitz SM
Haematologica 2023 Dec 1;108(12):3244-3260. doi: 10.3324/haematol.2023.282717. PMID: 38037801Free PMC Article
Hepatosplenic T-cell lymphoma: treatment challenges.
Bron D, De Leval L, Michiels S, Wittnebel S; EuroBloodNet for rare diseases
Curr Opin Oncol 2021 Sep 1;33(5):406-411. doi: 10.1097/CCO.0000000000000775. PMID: 34409955
Treatment of T-cell non-Hodgkin's lymphoma.
Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906

Recent clinical studies

Etiology

Biology and genetics of extranodal mature T-cell and NKcell lymphomas and lymphoproliferative disorders.
Lewis NE, Zhou T, Dogan A
Haematologica 2023 Dec 1;108(12):3261-3277. doi: 10.3324/haematol.2023.282718. PMID: 38037802Free PMC Article
Extranodal T- and NK-cell lymphomas.
de Leval L, Feldman AL, Pileri S, Nakamura S, Gaulard P
Virchows Arch 2023 Jan;482(1):245-264. Epub 2022 Nov 7 doi: 10.1007/s00428-022-03434-0. PMID: 36336765Free PMC Article
Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.
Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Challenges and new prospects in hepatosplenic γδ T-cell lymphoma.
Calvaruso M, Gulino A, Buffa S, Guarnotta C, Franco G, Cacciatore M, Bonura MG, Franco V, Florena AM
Leuk Lymphoma 2014 Nov;55(11):2457-65. Epub 2014 Mar 10 doi: 10.3109/10428194.2014.889821. PMID: 24506469
Peripheral T-cell lymphoma: the role of hematopoietic stem cell transplantation.
Gkotzamanidou M, Papadimitriou CA
Crit Rev Oncol Hematol 2014 Feb;89(2):248-61. Epub 2013 Sep 8 doi: 10.1016/j.critrevonc.2013.08.016. PMID: 24075060

Diagnosis

Extranodal T- and NK-cell lymphomas.
de Leval L, Feldman AL, Pileri S, Nakamura S, Gaulard P
Virchows Arch 2023 Jan;482(1):245-264. Epub 2022 Nov 7 doi: 10.1007/s00428-022-03434-0. PMID: 36336765Free PMC Article
Hepatosplenic T-cell lymphoma: a rare but challenging entity.
Pro B, Allen P, Behdad A
Blood 2020 Oct 29;136(18):2018-2026. doi: 10.1182/blood.2019004118. PMID: 32756940Free PMC Article
Hepatosplenic T-Cell Lymphomas.
Gowda L, Foss F
Cancer Treat Res 2019;176:185-193. doi: 10.1007/978-3-319-99716-2_9. PMID: 30596219
Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors.
Yabe M, Miranda RN, Medeiros LJ
Hum Pathol 2018 Apr;74:5-16. Epub 2018 Jan 11 doi: 10.1016/j.humpath.2018.01.005. PMID: 29337025
Hepatosplenic gamma-delta T-cell lymphoma.
Ferreri AJ, Govi S, Pileri SA
Crit Rev Oncol Hematol 2012 Aug;83(2):283-92. Epub 2011 Nov 1 doi: 10.1016/j.critrevonc.2011.10.001. PMID: 22047938

Therapy

Hepatosplenic T-Cell Lymphomas.
Gowda L, Foss F
Cancer Treat Res 2019;176:185-193. doi: 10.1007/978-3-319-99716-2_9. PMID: 30596219
Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.
Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Challenges and new prospects in hepatosplenic γδ T-cell lymphoma.
Calvaruso M, Gulino A, Buffa S, Guarnotta C, Franco G, Cacciatore M, Bonura MG, Franco V, Florena AM
Leuk Lymphoma 2014 Nov;55(11):2457-65. Epub 2014 Mar 10 doi: 10.3109/10428194.2014.889821. PMID: 24506469
Hepatosplenic gamma-delta T-cell lymphoma.
Ferreri AJ, Govi S, Pileri SA
Crit Rev Oncol Hematol 2012 Aug;83(2):283-92. Epub 2011 Nov 1 doi: 10.1016/j.critrevonc.2011.10.001. PMID: 22047938
Gamma-delta T-cell lymphomas.
Tripodo C, Iannitto E, Florena AM, Pucillo CE, Piccaluga PP, Franco V, Pileri SA
Nat Rev Clin Oncol 2009 Dec;6(12):707-17. Epub 2009 Nov 10 doi: 10.1038/nrclinonc.2009.169. PMID: 19901919

Prognosis

Hepatosplenic T-cell lymphoma: a rare but challenging entity.
Pro B, Allen P, Behdad A
Blood 2020 Oct 29;136(18):2018-2026. doi: 10.1182/blood.2019004118. PMID: 32756940Free PMC Article
Hepatosplenic T-Cell Lymphomas.
Gowda L, Foss F
Cancer Treat Res 2019;176:185-193. doi: 10.1007/978-3-319-99716-2_9. PMID: 30596219
Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors.
Yabe M, Miranda RN, Medeiros LJ
Hum Pathol 2018 Apr;74:5-16. Epub 2018 Jan 11 doi: 10.1016/j.humpath.2018.01.005. PMID: 29337025
Hepatosplenic gamma-delta T-cell lymphoma.
Ferreri AJ, Govi S, Pileri SA
Crit Rev Oncol Hematol 2012 Aug;83(2):283-92. Epub 2011 Nov 1 doi: 10.1016/j.critrevonc.2011.10.001. PMID: 22047938
Gamma-delta T-cell lymphomas.
Tripodo C, Iannitto E, Florena AM, Pucillo CE, Piccaluga PP, Franco V, Pileri SA
Nat Rev Clin Oncol 2009 Dec;6(12):707-17. Epub 2009 Nov 10 doi: 10.1038/nrclinonc.2009.169. PMID: 19901919

Clinical prediction guides

B-cell lymphoma-2 downregulation is a useful feature supporting a neoplastic phenotype in mature T-cell lymphomas.
Siddiqui F, Perez Silos V, Karube K, Yasin Goksu S, Nandakumar S, Saygin C, Onajin O, Prabu SS, Gurbuxani S, Arber DA, Tjota M, Segal J, Smith SM, Murga-Zamalloa CA, Venkataraman G
Hum Pathol 2022 Jul;125:48-58. Epub 2022 Apr 19 doi: 10.1016/j.humpath.2022.04.002. PMID: 35452696
Identification of Key Genes and Pathways Associated with Hepatosplenic T-Cell Lymphoma (HSTCL) by Bioinformatics Analysis.
Du J, Lu G, Xu B, Han XL, Mou WW
Clin Lab 2021 May 1;67(5) doi: 10.7754/Clin.Lab.2020.201018. PMID: 33978371
Hepatosplenic γδ T Cell Lymphoma Involving the Brain.
Iijima S, Chiba T, Maruyama K, Saito K, Kobayashi K, Yamagishi Y, Shibahara J, Takayama N, Shiokawa Y, Nagane M
World Neurosurg 2018 Oct;118:139-142. Epub 2018 Jul 18 doi: 10.1016/j.wneu.2018.07.048. PMID: 30030187
Cancer and immunomodulators in inflammatory bowel diseases.
Biancone L, Onali S, Petruzziello C, Calabrese E, Pallone F
Inflamm Bowel Dis 2015 Mar;21(3):674-98. doi: 10.1097/MIB.0000000000000243. PMID: 25545375
Splenic involvement by peripheral T-cell and NK-cell neoplasms.
Chan JK
Semin Diagn Pathol 2003 May;20(2):105-20. doi: 10.1016/s0740-2570(03)00013-3. PMID: 12945934

Recent systematic reviews

Systematic review: hepatosplenic T-cell lymphoma on biologic therapy for inflammatory bowel disease, including data from the Food and Drug Administration Adverse Event Reporting System.
Shah ED, Coburn ES, Nayyar A, Lee KJ, Koliani-Pace JL, Siegel CA
Aliment Pharmacol Ther 2020 Mar;51(5):527-533. Epub 2020 Jan 28 doi: 10.1111/apt.15637. PMID: 31990422Free PMC Article
A systematic review of factors that contribute to hepatosplenic T-cell lymphoma in patients with inflammatory bowel disease.
Kotlyar DS, Osterman MT, Diamond RH, Porter D, Blonski WC, Wasik M, Sampat S, Mendizabal M, Lin MV, Lichtenstein GR
Clin Gastroenterol Hepatol 2011 Jan;9(1):36-41.e1. Epub 2010 Oct 1 doi: 10.1016/j.cgh.2010.09.016. PMID: 20888436

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