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Lymphoepithelioid Variant Peripheral T-Cell Lymphoma, Not Otherwise Specified

MedGen UID:
301211
•Concept ID:
C1621719
•
Neoplastic Process
Synonyms: Lennert lymphoma; Lennert Lymphoma; Lennert Variant Peripheral T-Cell Lymphoma; Lennert's lymphoma; Lennert's Lymphoma; Lennert's Variant Peripheral T-Cell Lymphoma; Lymphoepithelioid Cell Variant Peripheral T-Cell Lymphoma; Lymphoepithelioid lymphoma; Lymphoepithelioid lymphoma (clinical); Lymphoepithelioid Variant Peripheral T-Cell Lymphoma; Lymphoepithelioid Variant Peripheral T-Cell Lymphoma, NOS
SNOMED CT: Lymphoepithelioid lymphoma (76481008); Lennert's lymphoma (76481008); Lymphoepithelioid lymphoma (clinical) (109976000); Lymphoepithelioid lymphoma (109976000); Lennert's lymphoma (109976000); Lennert lymphoma (76481008)

Definition

A variant of peripheral T-cell lymphoma, not otherwise specified. It is characterized by the presence of neoplastic small lymphocytes infiltrating the lymph nodes in a diffuse and less frequently interfollicular pattern. There is an associated proliferation of epithelioid histiocytes forming confluent clusters. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphoepithelioid Variant Peripheral T-Cell Lymphoma, Not Otherwise Specified

Recent clinical studies

Etiology

Expression of follicular helper T cell markers in nodal peripheral T cell lymphomas: a tissue microarray analysis of 162 cases.
Zhan HQ, Li XQ, Zhu XZ, Lu HF, Zhou XY, Chen Y
J Clin Pathol 2011 Apr;64(4):319-24. Epub 2011 Feb 17 doi: 10.1136/jcp.2010.084459. PMID: 21330314
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104
Nodal peripheral T-cell lymphomas and, in particular, their lymphoepithelioid (Lennert's) variant are often derived from CD8(+) cytotoxic T-cells.
Geissinger E, Odenwald T, Lee SS, Bonzheim I, Roth S, Reimer P, Wilhelm M, Müller-Hermelink HK, Rüdiger T
Virchows Arch 2004 Oct;445(4):334-43. Epub 2004 Jul 29 doi: 10.1007/s00428-004-1077-2. PMID: 15480768

Diagnosis

Clinicopathological diagnosis of Lennert lymphoma: a case report and review of the literature.
Ding S, Chen J, Su J, Liu J, Yin W, Qi F
Diagn Pathol 2024 Aug 16;19(1):111. doi: 10.1186/s13000-024-01533-x. PMID: 39148135Free PMC Article
Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature.
Yin Y, Liu H, Luo M, Yu G, Yin W, Li P
Diagn Pathol 2023 Feb 3;18(1):12. doi: 10.1186/s13000-023-01297-w. PMID: 36737805Free PMC Article
Rare Case of Double-Positive CD4/CD8 Immunophenotype in Lennert Lymphoma With Cutaneous Involvement: A Case Report.
Kim JH, Lee SK, Kim HY, Uh JA, Lee JH, Kim MS, Lee UH
Am J Dermatopathol 2022 Feb 1;44(2):121-125. doi: 10.1097/DAD.0000000000002011. PMID: 34816803
Expression of follicular helper T cell markers in nodal peripheral T cell lymphomas: a tissue microarray analysis of 162 cases.
Zhan HQ, Li XQ, Zhu XZ, Lu HF, Zhou XY, Chen Y
J Clin Pathol 2011 Apr;64(4):319-24. Epub 2011 Feb 17 doi: 10.1136/jcp.2010.084459. PMID: 21330314
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104

Therapy

Clinicopathological diagnosis of Lennert lymphoma: a case report and review of the literature.
Ding S, Chen J, Su J, Liu J, Yin W, Qi F
Diagn Pathol 2024 Aug 16;19(1):111. doi: 10.1186/s13000-024-01533-x. PMID: 39148135Free PMC Article
Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature.
Yin Y, Liu H, Luo M, Yu G, Yin W, Li P
Diagn Pathol 2023 Feb 3;18(1):12. doi: 10.1186/s13000-023-01297-w. PMID: 36737805Free PMC Article
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104

Prognosis

Clinicopathological diagnosis of Lennert lymphoma: a case report and review of the literature.
Ding S, Chen J, Su J, Liu J, Yin W, Qi F
Diagn Pathol 2024 Aug 16;19(1):111. doi: 10.1186/s13000-024-01533-x. PMID: 39148135Free PMC Article
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104

Clinical prediction guides

Clinicopathological diagnosis of Lennert lymphoma: a case report and review of the literature.
Ding S, Chen J, Su J, Liu J, Yin W, Qi F
Diagn Pathol 2024 Aug 16;19(1):111. doi: 10.1186/s13000-024-01533-x. PMID: 39148135Free PMC Article
Peripheral T-cell lymphoma--not otherwise specified.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA
Crit Rev Oncol Hematol 2011 Sep;79(3):321-9. Epub 2010 Aug 10 doi: 10.1016/j.critrevonc.2010.07.007. PMID: 20702104

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