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T-cell/histiocyte rich large B cell lymphoma

MedGen UID:
232943
•Concept ID:
C1321547
•
Neoplastic Process
Synonyms: Histiocyte-rich large B-cell lymphoma; T-cell histiocyte rich large B-cell lymphoma; T-cell hystiocyte rich large B-cell lymphoma; T-cell rich large B-cell lymphoma; T-cell rich/histiocyte-rich large B-cell lymphoma; T-Cell Rich/Histiocyte-Rich Large B-Cell Lymphoma; T-cell/histiocyte rich large B-cell lymphoma; T-Cell/Histiocyte Rich Lymphoma; T-cell/histiocyte rich lymphoma; T-Cell/Histiocyte-Rich Large B-Cell Lymphoma; T-cell/histiocyte-rich large B-cell lymphoma; THRLBCL
SNOMED CT: T-cell/histiocyte rich large B-cell lymphoma (450959001); T-cell rich large B-cell lymphoma (450959001); T-cell rich/histiocyte-rich large B-cell lymphoma (450959001); Histiocyte-rich large B-cell lymphoma (450959001); T-cell hystiocyte rich large B-cell lymphoma (724645006); T-cell histiocyte rich large B-cell lymphoma (724645006)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017597
Orphanet: ORPHA300857

Definition

T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL; see this term), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (see these terms) and has an aggressive clinical course. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVT-cell/histiocyte rich large B cell lymphoma
Follow this link to review classifications for T-cell/histiocyte rich large B cell lymphoma in Orphanet.

Professional guidelines

PubMed

Micronodular T-cell/histiocyte-rich large B-cell lymphoma of the spleen: histology, immunophenotype, and differential diagnosis.
Dogan A, Burke JS, Goteri G, Stitson RN, Wotherspoon AC, Isaacson PG
Am J Surg Pathol 2003 Jul;27(7):903-11. doi: 10.1097/00000478-200307000-00005. PMID: 12826882
T-cell/histiocyte-rich large B-cell lymphoma is a disseminated aggressive neoplasm: differential diagnosis from Hodgkin's lymphoma.
Fraga M, Sánchez-Verde L, Forteza J, García-Rivero A, Piris MA
Histopathology 2002 Sep;41(3):216-29. doi: 10.1046/j.1365-2559.2002.01466.x. PMID: 12207783
Hodgkin's disease: classification and differential diagnosis.
Harris NL
Mod Pathol 1999 Feb;12(2):159-75. PMID: 10071341

Recent clinical studies

Etiology

A Review of the Flow Cytometric Findings in Classic Hodgkin Lymphoma, Nodular Lymphocyte Predominant Hodgkin Lymphoma and T Cell/Histiocyte-Rich Large B Cell Lymphoma.
Ally F, Gajzer D, Fromm JR
Clin Lab Med 2023 Sep;43(3):427-444. Epub 2023 Jun 23 doi: 10.1016/j.cll.2023.04.011. PMID: 37481321
The utility of IMP3 immunohistochemical staining in differentiating nodular lymphocyte predominant Hodgkin Lymphoma from T-Cell/Histiocyte-Rich large B-Cell lymphoma.
Kosari F, Bakhshi T, Ameli F, Mokhtari M
BMC Cancer 2022 Dec 28;22(1):1359. doi: 10.1186/s12885-022-10321-z. PMID: 36577979Free PMC Article
Characteristics of diffuse large B-cell lymphoma in patients with primary Sjögren's syndrome.
Gorodetskiy VR, Probatova NA, Vasilyev VI
Int J Rheum Dis 2020 Apr;23(4):540-548. Epub 2020 Feb 25 doi: 10.1111/1756-185X.13800. PMID: 32100426Free PMC Article
Nodular lymphocyte predominant Hodgkin lymphoma: pathology, clinical course and relation to T-cell/histiocyte rich large B-cell lymphoma.
Hartmann S, Eichenauer DA
Pathology 2020 Jan;52(1):142-153. Epub 2019 Nov 28 doi: 10.1016/j.pathol.2019.10.003. PMID: 31785822
Clinical features and survival of patients with T-cell/histiocyte-rich large B-cell lymphoma: analysis of the National Cancer Data Base.
Ollila TA, Reagan JL, Olszewski AJ
Leuk Lymphoma 2019 Dec;60(14):3426-3433. Epub 2019 Jul 9 doi: 10.1080/10428194.2019.1639166. PMID: 31287335Free PMC Article

Diagnosis

Splenic Micronodular T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: The Corticosteroid Pretreatment Hypothesis.
Samueli B, Nalbandyan K, Benharroch D, Levi I
Acta Haematol 2022;145(3):310-317. Epub 2021 Nov 9 doi: 10.1159/000520791. PMID: 34753138Free PMC Article
Diffuse large B-cell lymphoma variants: an update.
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345
Diagnosis of Hodgkin lymphoma in the modern era.
Wang HW, Balakrishna JP, Pittaluga S, Jaffe ES
Br J Haematol 2019 Jan;184(1):45-59. Epub 2018 Nov 8 doi: 10.1111/bjh.15614. PMID: 30407610Free PMC Article
Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature.
Zheng SM, Zhou DJ, Chen YH, Jiang R, Wang YX, Zhang Y, Xue HL, Wang HQ, Mou D, Zeng WZ
World J Gastroenterol 2017 Jun 28;23(24):4467-4472. doi: 10.3748/wjg.v23.i24.4467. PMID: 28706431Free PMC Article
T cell/histiocyte-rich large B-cell lymphoma: an update on its biology and classification.
Tousseyn T, De Wolf-Peeters C
Virchows Arch 2011 Dec;459(6):557-63. Epub 2011 Nov 12 doi: 10.1007/s00428-011-1165-z. PMID: 22081105

Therapy

Unusual primary central nervous system T-cell/histiocyte-rich large B-cell lymphoma: a case report.
Toro J, Patiño J, Flórez A, Rivera JS
BMJ Case Rep 2022 Feb 8;15(2) doi: 10.1136/bcr-2021-246355. PMID: 35135790Free PMC Article
Splenic Micronodular T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: The Corticosteroid Pretreatment Hypothesis.
Samueli B, Nalbandyan K, Benharroch D, Levi I
Acta Haematol 2022;145(3):310-317. Epub 2021 Nov 9 doi: 10.1159/000520791. PMID: 34753138Free PMC Article
Clinical features and survival of patients with T-cell/histiocyte-rich large B-cell lymphoma: analysis of the National Cancer Data Base.
Ollila TA, Reagan JL, Olszewski AJ
Leuk Lymphoma 2019 Dec;60(14):3426-3433. Epub 2019 Jul 9 doi: 10.1080/10428194.2019.1639166. PMID: 31287335Free PMC Article
Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature.
Zheng SM, Zhou DJ, Chen YH, Jiang R, Wang YX, Zhang Y, Xue HL, Wang HQ, Mou D, Zeng WZ
World J Gastroenterol 2017 Jun 28;23(24):4467-4472. doi: 10.3748/wjg.v23.i24.4467. PMID: 28706431Free PMC Article
Splenic micronodular T-cell/histiocyte-rich large B-cell lymphoma: effect of prior corticosteroid therapy.
Kan E, Levy I, Benharroch D
Virchows Arch 2009 Oct;455(4):337-41. Epub 2009 Sep 9 doi: 10.1007/s00428-009-0830-y. PMID: 19763612

Prognosis

Spatial phenotyping of nodular lymphocyte predominant Hodgkin lymphoma and T-cell/histiocyte-rich large B-cell lymphoma.
Younes S, Subramanian A, Khan A, Zhao S, Binkley M, Natkunam Y
Blood Cancer J 2024 May 31;14(1):92. doi: 10.1038/s41408-024-01073-z. PMID: 38821935Free PMC Article
Nodular lymphocyte predominant Hodgkin lymphoma: pathology, clinical course and relation to T-cell/histiocyte rich large B-cell lymphoma.
Hartmann S, Eichenauer DA
Pathology 2020 Jan;52(1):142-153. Epub 2019 Nov 28 doi: 10.1016/j.pathol.2019.10.003. PMID: 31785822
Clinical features and survival of patients with T-cell/histiocyte-rich large B-cell lymphoma: analysis of the National Cancer Data Base.
Ollila TA, Reagan JL, Olszewski AJ
Leuk Lymphoma 2019 Dec;60(14):3426-3433. Epub 2019 Jul 9 doi: 10.1080/10428194.2019.1639166. PMID: 31287335Free PMC Article
Diagnosis of Hodgkin lymphoma in the modern era.
Wang HW, Balakrishna JP, Pittaluga S, Jaffe ES
Br J Haematol 2019 Jan;184(1):45-59. Epub 2018 Nov 8 doi: 10.1111/bjh.15614. PMID: 30407610Free PMC Article
Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature.
Zheng SM, Zhou DJ, Chen YH, Jiang R, Wang YX, Zhang Y, Xue HL, Wang HQ, Mou D, Zeng WZ
World J Gastroenterol 2017 Jun 28;23(24):4467-4472. doi: 10.3748/wjg.v23.i24.4467. PMID: 28706431Free PMC Article

Clinical prediction guides

The spectrum of nodular lymphocyte predominant Hodgkin lymphoma: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology.
Hartmann S, Dojcinov S, Dotlic S, Gibson SE, Hsi ED, Klapper W, Klimkowska M, Pinilla SMR, Richter J, Sabattini E, Tousseyn T, de Jong D
Virchows Arch 2023 Oct;483(4):451-463. Epub 2023 Aug 2 doi: 10.1007/s00428-023-03554-1. PMID: 37530790Free PMC Article
Spatial signatures identify immune escape via PD-1 as a defining feature of T-cell/histiocyte-rich large B-cell lymphoma.
Griffin GK, Weirather JL, Roemer MGM, Lipschitz M, Kelley A, Chen PH, Gusenleitner D, Jeter E, Pak C, Gjini E, Chapuy B, Rosenthal MH, Xu J, Chen BJ, Sohani AR, Lovitch SB, Abramson JS, Ishizuka JJ, Kim AI, Jacobson CA, LaCasce AS, Fletcher CD, Neuberg D, Freeman GJ, Hodi FS, Wright K, Ligon AH, Jacobsen ED, Armand P, Shipp MA, Rodig SJ
Blood 2021 Mar 11;137(10):1353-1364. doi: 10.1182/blood.2020006464. PMID: 32871584Free PMC Article
T-cell/histiocyte-rich large B-cell lymphoma of stomach.
Barut F, Kandemir NO, Gun BD, Ozdamar SO
J Pak Med Assoc 2016 Jul;66(7):905-7. PMID: 27427148
Cutaneous T-cell/histiocyte-rich B-cell lymphoma: a case report and review of the literature.
Vezzoli P, Fiorani R, Girgenti V, Fanoni D, Tavecchio S, Balice Y, Mozzana R, Crosti C, Berti E
Dermatology 2011;222(3):225-30. Epub 2011 Apr 30 doi: 10.1159/000327376. PMID: 21540569
Morphologic, immunphenotypic and clinical discriminators between T-cell/histiocyte-rich large B-cell lymphoma and lymphocyte-predominant Hodgkin lymphoma.
Mourad WA, Al Thani S, Tbakhi A, Al Omari M, Khafaga Y, Shoukri M, El Weshi A, Al Shabana M, Ezzat A
Hematol Oncol Stem Cell Ther 2008 Jan-Mar;1(1):22-7. doi: 10.1016/s1658-3876(08)50056-2. PMID: 20063524

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