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Joflaher/sandbox
Other nameslymphoepithelioid cell variant of the peripheral T-cell lymphoma not otherwise specified
SpecialtyOncology
Usual onsetAdults
TreatmentChemotherapy
Prognosisguarded
FrequencyRare

Lennert lymphoma, also termed lymphoepithelioid lymphoma or lymphoepithelioid variant of peripheral T-cell lymphoma,[1] is a rare subtype of the T cell lymphomas.[2] It was first characterized by Karl Lennert in 1952 as a variant of Hodgkin lymphoma based on the presence of cells resembling the Reed–Sternberg cells that typify Hodgkin lymphoma.[2][3] However, later studies concluded that these cells are not Reed-Sternberg cells and that Lennert lymphoma is not a variant of Hodgkin lymphoma.[4]

Lennert lymphoma is now regarded as one form of the peripheral T-cell lymphomas. The World Health Organization (2016) classified these peripheral T-cell lymphomas into more than 25 different subtypes such as the anaplastic large-cell lymphoma (including its ALK+ and ALK- subtypes), angioimmunoblastic T-cell lymphoma, and peripheral T-cell lymphoma not otherwise specified.[5][6] Peripheral T-cell lymphomas not otherwise specified include Lennert Lymphoma as well as several other lymphoma types.[6] However, the criteria used for diagnosing Lennert lymphomas has varied between different studies.[4][7][8][9] That is, Lennert lymphoma has been defined using a wide range of microscopic histologies and immunochemistries (i.e., proteins expressed by tumor cells). Descriptions of the clinical features of and survival times for Lennert lympomohas have also varied across different reports.[10] Here, Lennert lymphoma is reviewed based on studies that future criteria may find are indicative of another type of lymphoma. Studies that question the identity of the "Lennert lymphomas" diagnosed by other studies are included in this review.

Clinical presentation

In a review study of 26 patients (i.e., 5 men and 11 women, median age 72 years, range from 44 to 90 years) diagnosed with Lennert lymphoma: a) 7 presented with B symptoms (i.e., fever, night sweats, and/or unintentional weight loss); b) 8 presented with (or later acquired) cancer cells in their bone marrow, blood, skin, liver, lung, bone, and/or subcutaneous tissue in addition to their lymph nodes; c) 6 presented with hepatosplenomegaly (i.e., enlargement of both the liver and spleen); d) 5 had a 2 or 3 score on the ECOG/WHO/Zubrod questionnaire (i.e., they stayed in bed some or most of the day) at presentation; and e) 16 had a 3 or 4 on the International Prognostic Index (i.e., they had a projected 5 year survival of 43% or less) at presentation.[10] The prognosis of Lennert lymphomas have also been estimated using the same Ann Arbor staging used for all non-Hodgkin lymphomas: Stage I, involvement of a lymph node region or a single set of lymph nodes or other tissue in the lymphatic system; Stage II, involvement of lymph node, other tissue in the lymphatic system, and/or non-lymphatic tissue all of which are on the same side of the diaphragm; Stage III, involvement of lymph nodes, othert tissue in the lymphoid system, and/or non-lymphoid tissues with at least one of the cites being on the opposite side of the diaphragm from the other sites; Stage IV, disseminated disease occurring on both sides of the diaphragm and involving one or more non-lymphatic organs or all cases in which the liver and/or bone marrow is/are involved.[11] Among the 26 patients in the review study, 2 had not been staged, 1 had Stage I, 5 had Stage II, 9 had Stage III, 8 had stage IV, and 1 had stage III or IV disease.[10] A later study[6] of 10 patients (9 males, 1 female; mean age of 48.7, range 22–67 years) reported that: 1 presented with B sypmtoms, 1 presented with bone marrow involvement, 2 presented with involvement of tissues outside of their lymph nodes, 3 had bulky disease; and 4 had a mediastinal tumor that was not further defined. A 2009 review reported on one patient and 11 previously reported patients with Lennert lymphomas that invaded their skin. The skin lesions were asymptomatic, non-ulcerated, and red to violet papules, nodules, or small plaques on the trunk and extremities.[1] In a more recent report, one patient with Lennert lymphoma had symptomatic skin lesions: these patches itched, felt warm, were located on the nape of the neck, back, and buttocks, and on microscopic examination were infiltrated with Lennert lymphoma cells.[12] Another recent study reported that a 65 year old patient had a painless subcutaneous soft tissue Lennert lymphoma on his forehead but no evidence that it had invaded the nearby bone or skin surface nor that it was present in his lymph nodes, It was suggested that this Lennert lymphoma originated in the subcutaneous soft tissue rather than lymphatic system.[4]

Follicular helper T cell bearing lymphoma

In the study of 26 patients with Lennert lymphoma, 15 had cells that expressed one or more of the three proteins expressed by follicular helper T cells viz., PD-1, CXCL13, and CD10. These patients had a more severe form of Lennert lymphoma as defined by there survival times: the percentage of patients surviving 52 months after the diagnosis in the 15 patients with tumor cells that had one or more of these marker proteins was 35%, i.e., 5 of 15 patients, while it was 73%, i.e., 8, of the 11 patients, that lacked tumor cells with one or more of these maker proteins. This difference was statistically significant (p-value of 0.011) based on the log-rank testing and Kaplan–Meier estimator for the survival curves of the two patient groups.[9][10] It should be noted, however, that other studies suggest that "Lennert lymphomas" with features of follicular helper T cell-bearing lymphomas are best defined as follicular helper T-cell lymphomas[6] or angioimmunoblastic T cell lymphoma[13] rather than Lennert lymphomas.

Human T-cell lymphotropic virus type 1

Globally, the number of individuals infected with the human T-cell lymphotropic virus type 1 is estimated to be 5-10 million.[14] These individuals reside principally in this virus's endemic areas, i.e., Japan (mainly the islands of Kyushu and Shikoku), sub-Saharan Africa, South America, the Caribbean area, Iran, Romania, and Melanesia.[15] This virus causes adult T-cell leukemia/lymphoma, tropical spastic paraparesis, and various inflammatory disorders such as uveitis and dermatitis.[14] Clinically and histopathologically, T-cell leukemia/lymphoma mimics and is therefore misdiagnosed as other types of T-cell neoplasms including Lennert lymphoma.[11] One large study suggested that in diagnosing Lennert lymphomas test be conducted to determine the presence of the human T-cell lymphotropic virus type 1, particularly in individuals living in or from this virus's endemic areas. The presence of this virus in Lennert lymphoma-like tissues indicates that the lymphoma is an adult T-cell leukemia/lymphoma[15]

Epstein-Barr virus

The Epstein-Barr virus infects about 95% of the world's population. It initially causes infectious mononucleosis, non-specific symptoms, or no symptoms but nonetheless enters a latency period in its hosts and thereafter may become active in causing wide range of lymphoproliferative diseases termed Epstein–Barr virus–associated lymphoproliferative diseases[16] as well as various other diseases (see The role of EBV in disease). An early study reported that 11 of 15 patients expressed Epstein-Barr virus DNA in their Lennert lymphoma tissues.[17] Other studies have reported that this virus is less prevalent in Lennert lymphomas. For example, more recent studies have reported that 8 of 26 patients,[10] 4 of 13 patients,[11] and none of 6 patients[18] expressed the products made by the Epstein-Barr virus in their Lennert lymphoma tissues. The significance of the presence of the Epstein-Barr virus in Lennert lymphoma is unclear: studies have suggested that its presence in Lennert lymphoma cells is probably not directly involved in the development or severity[17] or is associated with a more severe form of this lymphoma.[18]

Histopathology

The microscopic examination of Lennert lymphoma tissues commonly shows numerous granuloma-like aggregates of epithelioid histiocytes[2] intermingled with varying numbers of T cells and B cells (i.e., subtypes of lymphocytes).[10] However, some cases of Lennert lymphomas consist of: a) epithelioid histiocytes intermingled with small, similarly appearing, lymphoid cells but without distinct granuloma or b) abundant histiocytic clusters and medium to large atypical lymphoid cells. In one study, more than 90% of the T cells in the Lennert lymphoma tissues expressed Cd3, i.e., a protein complex that is a co-receptor on T cells.[2] In the study of 26 patients with Lennert lymphomas, all 26 patients' lymphomas had CD3-expressing cells, 24 had CD4-expressing cells, and 5 had CD8-expressing cells.[10] CD4 is a membrane glycoprotein receptor expressed on helper T-cells; and CD8 is a transmembrane glycoprotein co-receptor for the T-cell receptor on T cells.[2][4][10][19][5]

Cytogenetics

Lennert lymphoma tissue samples from 15 patients were cultured and the chromosomes in metaphase were analyzed using a standard G banding protocol. The lymphoma tissues of 7 patients exhibited a normal karyotype, i.e., a normal appearing and complete set of chromosomes. However, eight patients' lymphoma tissues had various chromosome cytogenetic abnormalities: two had trisomy of chromosome 7 plus an extra X chromosome, one had trisomy of chromosome 3 and chromosome 5, one had trisomy of chromosome 3 and chromosome 7 plus an additional X chromosome, one had trisomy of chromosome 3, and three had additions and/or deletions to parts of multiply chromosomes.[10] An analysis of pre-selected genes found 455 genes that expressed different levels of these genes' RNA or protein products in the tumor tissues of Lennert lymphoma versus the group of non-Lennert peripheral T-cell lymphoma not otherwise specified tumor tissues: 385 of these products were more highly express in Lennert lymphoma than the group of non-Lennert peripheral T-cell lymphoma not otherwise specified tumor tissues. These findings, if confirmed in other studies, would be extremely helpful in making the diagnosis of Lennert lymphoma.[6]

Cancer cells in Lennert lymphoma

A 1986 study cited 11 publications which suggested that the cancer cells in Lennert lymphoma were T cells. The study agreed with this suggestion based on finding that their patient with Lennert lymphoma had a small portion of tumor tissue T cells were hypotetraploid, i.e., missing one or more chromosomes. The study suggested that this small percentage of T cells were the malignant cells in Lennert lymphoma.[20] In the same year, a study co-authored by Karl Lennert found that the T4-expressing lymphocytes but not the other cells types in the Lennert lymphoma tissues of four patients were rapidly proliferating (i.e., rapidly growing and multiplying) based on their levels of the cell proliferation marker Ki-67). This study also found that some of the these rapidly proliferating lymphocytes had morphological features found in cancerous lymphocytes. Based on these finding, the study suggested that the rapidly proliferating, T4-expressing T cells are the cancer cells in Lennert lymphomas.[2] Another study also suggested that CD4-expressing T cells were the cancer cells in Lennert lymphomas based on their being the most prevalent T cells in the tumor tissues of 8 of 12 Lennert lymphoma cases.[6] While studies agree that T cells are the malignant cell in Lennert lymphoma, the exact type of T cell that is cancerous in Netter lymphoma is controversial. Initial studies proposed the the malignant cells in Lennert Lymphoma were CD4-expressing T helper cells whereas other studies have proposed that cytotoxic T cells are the malignant cells in this disease.[4]

Diagnosis

Lennert lymphoma has been diagnosed based primarily on its histopathology and immunohistochemistry (e.g., the expression of certain proteins such as CD3, CD4, and CD8 in the tumor cells).[6] However, different studies have used different histopathology and immunochemistry criteria in diagnosing this lymphoma.[19][5]

rapid proliferation of small lymphocytesPMID: 19775391 proliferation of small-sized atypical lymphocytes PMID: 36737805 Our results strongly suggest that Lennert's lymphoma is a CD4-positive T cell lymphomaPMID: 2943330

characterized by numerous aggregates of epithelioid histiocytes intermingled with atypical T lymphocytes.[10]

The small lymphoid cells were positive for CD3, CD4, and CD5 and were negative for CD20, CD8, and CD10 (Figure 2B). The Ki-67 proliferative index was 60% in t[19]

epithelioid cells expressing histiocyte antigens and large numbers of T cells CD8 + lymphocytes More than 90% of the T cells expressed the T cell antigens CD3, CD5, and CD2, and the majority of lymphoid cells (>80%) expressed CD4, whereas three of the four samples were composed of less than 10% CD8-positive cells .[2]

In our case the cytologic findings were similar to those found in other peripheral T-cell lymphomas [21]

Prognosis

See also

References

  1. ^ a b Summers TA, Rush W, Aguilera N, Lupton G (October 2009). "Cutaneous involvement in the lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified (Lennert lymphoma). Report of a case and review of the literature". Journal of Cutaneous Pathology. 36 Suppl 1: 25–30. doi:10.1111/j.1600-0560.2008.01203.x. PMID 19775391.
  2. ^ a b c d e f g Feller AC, Griesser GH, Mak TW, Lennert K (September 1986). "Lymphoepithelioid lymphoma (Lennert's lymphoma) is a monoclonal proliferation of helper/inducer T cells". Blood. 68 (3): 663–7. PMID 2943330.
  3. ^ Lennert K: Zur Histologischen Diagnose der Lymphogranulomatose. Frankfurt, FRG, Habil-Schrift, 1952
  4. ^ a b c d e Yin Y, Liu H, Luo M, Yu G, Yin W, Li P (February 2023). "Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature". Diagnostic Pathology. 18 (1): 12. doi:10.1186/s13000-023-01297-w. PMC 9896693. PMID 36737805.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  5. ^ a b c Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA (September 2011). "Peripheral T-cell lymphoma--not otherwise specified". Critical Reviews in Oncology/hematology. 79 (3): 321–9. doi:10.1016/j.critrevonc.2010.07.007. PMID 20702104.
  6. ^ a b c d e f g Etebari M, Navari M, Agostinelli C, Visani A, Peron C, Iqbal J, Inghirami G, Piccaluga PP (2019). "Transcriptional Analysis of Lennert Lymphoma Reveals a Unique Profile and Identifies Novel Therapeutic Targets". Frontiers in Genetics. 10: 780. doi:10.3389/fgene.2019.00780. PMC 6748072. PMID 31552092.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  7. ^ Kitamura A, Yamashita Y, Sato Y, Hasegawa Y, Kojima H, Nagasawa T, Mori N (October 2005). "Aggressive Lennert's lymphoma: report of three cases in comparison to non-aggressive Lennert's lymphoma". Pathology International. 55 (10): 626–31. doi:10.1111/j.1440-1827.2005.01880.x. PMID 16185292.
  8. ^ Hartmann S, Agostinelli C, Klapper W, Korkolopoulou P, Koch K, Marafioti T, Piccaluga PP, Patsouris E, Pileri S, Hansmann ML (December 2011). "Revising the historical collection of epithelioid cell-rich lymphomas of the Kiel Lymph Node Registry: what is Lennert's lymphoma nowadays?". Histopathology. 59 (6): 1173–82. doi:10.1111/j.1365-2559.2011.04069.x. PMID 22175897.
  9. ^ a b Ondrejka SL, Amador C, Climent F, Ng SB, Soma L, Zamo A, Dirnhofer S, Quintanilla-Martinez L, Wotherspoon A, Leoncini L, de Leval L (September 2023). "Follicular helper T-cell lymphomas: disease spectrum, relationship with clonal hematopoiesis, and mimics. A report of the 2022 EA4HP/SH lymphoma workshop". Virchows Archiv : an International Journal of Pathology. 483 (3): 349–365. doi:10.1007/s00428-023-03607-5. PMC 10541838. PMID 37500795.
  10. ^ a b c d e f g h i j Kurita D, Miyoshi H, Yoshida N, Sasaki Y, Kato S, Niino D, Sugita Y, Hatta Y, Takei M, Makishima M, Ohshima K (September 2016). "A Clinicopathologic Study of Lennert Lymphoma and Possible Prognostic Factors: The Importance of Follicular Helper T-cell Markers and the Association With Angioimmunoblastic T-cell Lymphoma". The American Journal of Surgical Pathology. 40 (9): 1249–60. doi:10.1097/PAS.0000000000000694. PMID 27428734.
  11. ^ a b c Weisenburger DD, Savage KJ, Harris NL, Gascoyne RD, Jaffe ES, MacLennan KA, Rüdiger T, Pileri S, Nakamura S, Nathwani B, Campo E, Berger F, Coiffier B, Kim WS, Holte H, Federico M, Au WY, Tobinai K, Armitage JO, Vose JM (March 2011). "Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project". Blood. 117 (12): 3402–8. doi:10.1182/blood-2010-09-310342. PMID 21270441.
  12. ^ Kim JH, Lee SK, Kim HY, Uh JA, Lee JH, Kim MS, Lee UH (February 2022). "Rare Case of Double-Positive CD4/CD8 Immunophenotype in Lennert Lymphoma With Cutaneous Involvement: A Case Report". The American Journal of Dermatopathology. 44 (2): 121–125. doi:10.1097/DAD.0000000000002011. PMID 34816803.
  13. ^ Zhan HQ, Li XQ, Zhu XZ, Lu HF, Zhou XY, Chen Y (April 2011). "Expression of follicular helper T cell markers in nodal peripheral T cell lymphomas: a tissue microarray analysis of 162 cases". Journal of Clinical Pathology. 64 (4): 319–24. doi:10.1136/jcp.2010.084459. PMID 21330314.
  14. ^ a b Seighali N, Shafiee A, Rafiee MA, Aminzade D, Mozhgani SH (May 2023). "Human T-cell lymphotropic virus type 1 (HTLV-1) proposed vaccines: a systematic review of preclinical and clinical studies". BMC Infectious Diseases. 23 (1): 320. doi:10.1186/s12879-023-08289-7. PMC 10173209. PMID 37170214.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  15. ^ a b Khanlari M, Ramos JC, Sanchez SP, Cho-Vega JH, Amador A, Campuzano-Zuluaga G, Vega F, Chapman JR (July 2018). "Adult T-cell leukemia/lymphoma can be indistinguishable from other more common T-cell lymphomas. The University of Miami experience with a large cohort of cases". Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc. 31 (7): 1046–1063. doi:10.1038/s41379-018-0037-3. PMC 6931282. PMID 29449683.
  16. ^ Rezk SA, Zhao X, Weiss LM (June 2018). "Epstein–Barr virus–associated lymphoid proliferations, a 2018 update". Human Pathology. 79: 18–41. doi:10.1016/j.humpath.2018.05.020. PMID 29885408. S2CID 47010934.
  17. ^ a b Anagnostopoulos I, Hummel M, Tiemann M, Korbjuhn P, Parwaresch MR, Stein H (October 1994). "Frequent presence of latent Epstein-Barr virus infection in lymphoepithelioid cell lymphoma (Lennert's lymphoma)". Histopathology. 25 (4): 331–7. doi:10.1111/j.1365-2559.1994.tb01351.x. PMID 7835838.
  18. ^ a b Jeon YK (July 2016). "Clinicopathological analysis of a case series of peripheral T-cell lymphomas, not otherwise specified, of lymphoepithelioid variant (Lennert's lymphoma). A Central European single-center study-reply". Human Pathology. 53: 194–5. doi:10.1016/j.humpath.2015.12.034. PMID 27016487.
  19. ^ a b c Cho U, Park G, Kim JA, Im S (February 2021). "Lymphoepithelioid variant of peripheral T cell lymphoma (Lennert lymphoma): Cytologic and histologic features". Diagnostic Cytopathology. 49 (2): 322–324. doi:10.1002/dc.24552. PMID 32745376.
  20. ^ Stonesifer KJ, Benson NA, Ryden SE, Pawliger DF, Braylan RC (August 1986). "The malignant cells in a Lennert's lymphoma are T lymphocytes with a mature helper surface phenotype. A multiparameter flow cytometric analysis". Blood. 68 (2): 426–9. PMID 2942199.
  21. ^ Vaíllo Vinagre A, Gutiérrez Martín A, Pérez Barrios A, Alberti Masgrau N, Ruiz Liso JM (2004). "Lymphoepithelioid cell lymphoma (Lennert's lymphoma). Report of a case with fine needle aspiration cytology". Acta Cytologica. 48 (2): 234–8. doi:10.1159/000326323. PMID 15085759.
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