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ABSTRACT. Peixoto T.C., de Freitas J.L., Farias S.S., Vieira Filho C.H., Larangeira D.F., Mascarenhas M.B., Nogueira V.A. & Barrouin-Melo S.M. [Primary heart lymphoma associated with visceral leishmaniasis in a dog - Case report.] Linfoma primário cardíaco associado à leishmaniose visceral em cão - Relato de Caso. Revista Brasileira de Medicina Veterinária 38(Supl.1):47-54, 2016. Departamento de Anatomia, Patologia e Clínicas Veterinárias, Escola de Medicina Veterinária e Zootecnia, Universidade Federal da Bahia, Av. Adhemar de Barros, 500, Ondina, Salvador, BA 40170-110, Brasil. E-mail: firstname.lastname@example.org It describes the clinicopathological and immunohistochemical aspects of a fatal primary heart T-cell lymphoma in a dog presenting visceral leishmaniasis (VL), from an endemic area in the State of Bahia. A female dog, Pitbull, with 2-year-old was seen at internal medicine section Veterinary Hospital (HOSPMEV) of the Universidade Federal da Bahia, with a history of progressive chronic skin disease with pruritus at variable score. The owner returned six months after the first review reporting the dog had appetite loss, oliguria, weight loss and dyspnea. Indirect ELISA for detection of anti-Leishmania antibodies had given positive results and parasitological examination of splenic aspirate evidenced amastigotes of Leishmania sp. The dog presented cardiorespiratory falence in HOSPMEV and evoluted to death. At macroscopic examination of the heart, it was found epicardium irregular surface due to the presence of multiple areas whitish homogeneous, sometimes protruding located in all four heart compartments. At incision, there were multiple nodules or masses of irregular shape and varying size. Additionally, superficial lymph nodes were moderately increased in size. Histopathological evaluation showed in the heart and diaphragm, atypical proliferation of large or medium-sized lymphoid cells with round or ovoid nuclei, sometimes cleaved, hyperchromatic, sometimes vesicular, with evident nucleoli and scarce amphophilic cytoplasm, arranged in diffuse mantle (solid pattern) or groups of large agglomerates between cardiac muscle fibers. Spleen, lymph nodes and bone marrow evidenced lots of amastigotes of Leishmania sp. within the cytoplasm of macrophages in the interstitium or free. Coexistence between infection with Leishmania and cancer has been observed in humans and animals, and a number of epidemiological, experimental and laboratory studies suggest an association between these two entities in human. It is indicative that the dysfunction of the immune system caused by Leishmania infection can make organism more susceptible to express an unusual neoplastic disease, as the present report.