Abstract
Extranodal T-lymphoproliferative disorders or T-cell lymphomas (TLPD) are classified according to the WHO Classification (4th and upcoming 5th editions) (Swerdlow et al., IARC Press 1; Alaggio et al., Leukemia 36(7):1720–1748, 2) and to the International Consensus Classification Update (Campo et al., Blood 140(11):1229–1253, 3) upon several morphologic, phenotypic, and genetic features. None of those at present included has been characterized by primary pulmonary onset. We herein present two such cases which, to the best of our knowledge, have not been previously reported and that might represent another variant of T-cell proliferation at mucosal sites. The two cases share similar histological and phenotypic features, suggesting an origin from CD4 + effector memory T cells with the expression of a CD279/PD-1 antigen. They are both monoclonal, harbor few mutations, and show no disease progression outside the lung. They only differ concerning the local extension of the process and clinical setting. The two cases are examples of so far unreported primary pulmonary TLDP, with limited stage and low proliferative index. A possible relationship with a local yet unknown inflammatory trigger that might have favored the development of the T-cell clone cannot be ruled out.
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Funding
The study was supported by Associazione Italiana Leucemie Linfomi e Mieloma (AIL), Bologna, Italy, and by the 5 × 1000AIRC grant no. 21198 to Pileri S.
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E.S. designed the study and wrote and revised the manuscript; S.P. supported and revised the manuscript; F.A. and C.R. were involved in both cases’ diagnosis and wrote the first manuscript draft; C.B. collected information. C.A. revised the manuscript; A.B. provided clinical correlation; S.R., A.G., C.M., E.O., and C.T. performed ancillary studies in Bologna; and F.M. and G.M. performed ancillary studies in Milan.
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Sabattini, E., Bertuzzi, C., Broccoli, A. et al. Primary pulmonary T-cell lymphoproliferative disorders with a limited-stage, low proliferative index, and unusual clinical behavior: two cases of a rare occurrence. Virchows Arch 482, 899–904 (2023). https://doi.org/10.1007/s00428-022-03455-9
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DOI: https://doi.org/10.1007/s00428-022-03455-9