Coexistence of T-Large Granular Lymphocyte Leukemia and Peripheral T Cell Lymphoma-NOS with indolent behaviour

Main Article Content

Luca Guarnera
Valentina Boldrini
Gianmario Pasqualone
Carolina Cimino
Elisa Meddi
Roberta Laureana
Donata Trivigno
Giovanni Del Poeta
Alessandro Mauriello
Lucia Anemona
Massimiliano Postorino
Maria Cantonetti

Keywords

PTCL-NOS, LGLL, Aggressive T-cell lymphomas, Indolent T-cell lymphomas

Abstract

T-cell lymphomas and leukemias are highly heterogeneous groups of rare disorders. We report a case of a 68-year-old man patient who develops two different T-cell neoplasms (Large Granular Lymphocyte Leukemia [LGLL] in 2018 and Peripheral T-cell non-Hodgkin lymphoma  not otherwise specified [PTCL-NOS] in 2019) with a previous diagnosis of B-cell marginal zone lymphoma in 2010, treated with two lines of chemo-immunotherapy. The coexistence of these different T-cell neoplasms is rarely reported in literature and, moreover, is usually described as an LGLL transformation into PTCL-NOS; differently from these examples, herein the simultaneous conditions appear to be driven by different T-cell clones. Furthermore, the PTCL-NOS had a quite unusual behaviour, with a good disease control without intensive treatment. Because of these features, it could belong to a subgroup of indolent PTCL-NOS, not yet described in the WHO classification of T-cell neoplasms, which could benefit of less aggressive treatment.

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References

[1] Jevremovic D, Olteanu H. Flow Cytometry Applications in the Diagnosis of T/NK-Cell Lymphoproliferative Disorders. Cytometry Part B - Clinical Cytometry 2019. https://doi.org/10.1002/cyto.b.21768.
[2] Pizzi M, Margolskee E, Inghirami G. Pathogenesis of Peripheral T Cell Lymphoma. Annual Review of Pathology: Mechanisms of Disease 2018. https://doi.org/10.1146/annurev-pathol-020117-043821.
[3] Lamy T, Moignet A, Loughran TP. LGL leukemia: From pathogenesis to treatment. Blood 2017. https://doi.org/10.1182/blood-2016-08-692590.
[4] Barilà G, Calabretto G, Teramo A, Vicenzetto C, Gasparini VR, Semenzato G, et al. T cell large granular lymphocyte leukemia and chronic NK lymphocytosis. Best Practice and Research: Clinical Haematology 2019. https://doi.org/10.1016/j.beha.2019.06.006.
[5] Teramo A, Barilà G, Calabretto G, Ercolin C, Lamy T, Moignet A, et al. STAT3 mutation impacts biological and clinical features of T-LGL leukemia. Oncotarget 2017. https://doi.org/10.18632/oncotarget.18711.
[6] Broccoli A, Zinzani PL. Peripheral T-cell lymphoma, not otherwise specified. Blood 2017. https://doi.org/10.1182/blood-2016-08-692566.
[7] Swerdlow SH, Campo E, Pileri SA, Lee Harris N, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016. https://doi.org/10.1182/blood-2016-01-643569.
[8] d’Amore F, Gaulard P, Trümper L, Corradini P, Kim WS, Specht L, et al. Peripheral T-cell lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology 2015. https://doi.org/10.1093/annonc/mdv201.
[9] Lee J, Park K, Kim KH, Bang HI, Yoon SY, Choi IH. Diagnostic challenges of indolent peripheral T cell lymphoma: A case report and literature review. Medicine 2020. https://doi.org/10.1097/MD.0000000000022657.
[10] Penn I. The role of immunosuppression in lymphoma formation. Springer Seminars in Immunopathology 1998. https://doi.org/10.1007/s002810050040.
[11] Nijland ML, Koens L, Pals ST, Ten Berge IJM, Bemelman FJ, Kersten MJ. Clinicopathological characteristics of T-cell non-Hodgkin lymphoma arising in patients with immunodeficiencies: A single-center case series of 25 patients and a review of the literature. Haematologica 2018. https://doi.org/10.3324/haematol.2017.169987.
[12] Savola P, Rajala H, Mustjoki S. LGL leukemia and autoimmunity - the borderline between autoimmune disease and cancer is becoming blurred. Duodecim; Laaketieteellinen Aikakauskirja 2016.
[13] Yoshida N, Nishikori M, Izumi T, Imaizumi Y, Sawayama Y, Niino D, et al. Primary peripheral T-cell lymphoma, not otherwise specified of the thyroid with autoimmune thyroiditis. British Journal of Haematology 2013. https://doi.org/10.1111/bjh.12255.
[14] Perry AM, Warnke RA, Hu Q, Gaulard P, Copie-Bergman C, Alkan S, et al. Indolent T-cell lymphoproliferative disease of the gastrointestinal tract. Blood 2013. https://doi.org/10.1182/blood-2013-07-512830.
[15] Matutes E, Wotherspoon AC, Parker NE, Osuji N, Isaacson PG, Catovsky D. Transformation of T-cell large granular lymphocyte leukaemia into a high-grade large T-cell lymphoma. British Journal of Haematology 2001. https://doi.org/10.1046/j.1365-2141.2001.03220.x.
[16] Belhadj M, Mansour D, Kaltenbach S, Deau-Fischer B, Franchi P, Tamburini J, et al. T-cell large granular lymphocyte leukemia transfomation into aggressive t-cell lymphoma: A report of two cases with molecular characterization. Haematologica 2019. https://doi.org/10.3324/haematol.2018.205542.
[17] Nakmaura N, Carreras J, Kikuti YY, Miyaoka M, Tomita S, Ishida F. RICHTER TRANSFORMATION OF T-CELL LARGE GRANULAR CELL LEUKEMIA. Hematological Oncology 2017. https://doi.org/10.1002/hon.2439_164.
[18] Hayashi E, Takata K, Sato Y, Tashiro Y, Tachiyama Y, Sawada-Kitamura S, et al. Distinct morphologic, phenotypic, and clinical-course characteristics of indolent peripheral T-cell lymphoma. Human Pathology 2013. https://doi.org/10.1016/j.humpath.2013.03.002.