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Vadim R Gorodetskiy,1 Natalya A Probatova,2 Natalia A Kupryshina,3 Svetlana G Palshina,1 Tatiana N Obukhova,4 Yulia V Sidorova,5 Natalya V Ryzhikova,5 Andrey B Sudarikov5 1Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia; 2Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Moscow, Russia; 3Hematopoiesis Immunology Laboratory, N.N. Blokhin Russian Cancer Research Center, Moscow, Russia; 4Cytogenetic Laboratory, National Research Center for Hematology, Moscow, Russia; 5Laboratory of Molecular Hematology, National Research Center for Hematology, Moscow, RussiaCorrespondence: Vadim R GorodetskiyDepartment of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Kashirskoye Shosse 34А, Moscow 115522, RussiaTel +7 916 517-81-92Fax +7 499 6144468Email gorodetskiyblood@mail.ruAbstract: The peculiar features of T-cell large granular lymphocytic leukemia (T-LGLL) are its association with autoimmune disorders (particularly with rheumatoid arthritis (RA)) and a broad spectrum of B-cell lymphoproliferative disorders. However, association of T-LGLL with mantle cell lymphoma (MCL) is extremely rare. Here, we describe a case of an 80-year-old man admitted with suspected Felty’s syndrome. The blood count showed white blood cells at 2.2× 109/L, with 3% neutrophils, 88% lymphocytes, and at 0.66× 109/L LGLs. The spleen had been removed 43 months prior to the admission due to suspected B-cell splenic lymphoma. Re-examination of the spleen revealed cyclin D1+ and SOX11− lymphocytes in the inner part of the unexpanded mantle zones of the white pulp follicles, thus displaying a so-called in situ histologic pattern of MCL, and in small clusters in the red pulp. The splenic cords were moderately expanded by lymphocytes expressing CD3, TIA1, and granzyme B but not CD4 and CD8. Monoclonal rearrangements of the immunoglobulin heavy chain gene and the T-cell receptor (TCR) gamma and delta chain genes, polyclonal rearrangements of the TCR beta chain gene, mutation of the signal transducer and activator of transctiption 3 gene (c.1940A>T; p.N647I), and t(11;14)(q13;q32) translocation were identified in the spleen sample. Flow cytometry of bone marrow revealed a population of TCR γδ+, CD3+, CD4−, CD5−, CD7+, CD8−, CD16−, CD56−, and CD57− lymphocytes. Fragment analysis demonstrated identical TCR gene clonal rearrangement patterns in the spleen and bone marrow samples. In this study, we describe the first case of simultaneous presentation of γδ T-LGLL and leukemic non-nodal MCL (L-NN-MCL) in a patient with RA and present morphological findings of L-NN-MCL in the spleen.Keywords: composite lymphoma, spleen, mantle cell lymphoma, in situ, large granular lymphocyte leukemia, rheumatoid arthritis