RESUMEN
Large granular lymphocyte leukemia (LGL) is a clonal, lymphoproliferative disorder with an indolent disease course. T-cell LGL (T-LGL) is the most common type of LGL driven from T-cell lineage (85%). The coexistence of T-LGL with several types of autoimmune disorders, mostly rheumatoid arthritis (RA), has been reported. Felty's syndrome (FS) is defined by splenomegaly, low neutrophil count, and destructive arthritis and is usually seen in <1% of patients with RA. About 30% to 40% of patients with FS have been reported to have an expansion of large granulated lymphocytes in the circulation. FS and T-LGL are similar in terms of clinical manifestations, response to immunosuppressive therapy, their smoldering course, and immunogenetic findings, proposing FS and T-LGL with RA might be different aspects of a single disease spectrum. In this article, we present a case with long-standing RA who had never been on DMARD (Disease Modifying Anti-Rheumatic Drugs) treatment found to have constitutional symptoms, neutropenia, and splenomegaly, and the patient was diagnosed with T-LGL.
Asunto(s)
Artritis Reumatoide/complicaciones , Síndrome de Felty/complicaciones , Leucemia Linfocítica Granular Grande/diagnóstico , Humanos , Leucemia Linfocítica Granular Grande/complicaciones , Leucemia Linfocítica Granular Grande/inmunología , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Neutropenia/complicaciones , Esplenomegalia/complicaciones , Linfocitos T/inmunologíaRESUMEN
Glomus tumors are mesenchymal neoplasms derived from glomus bodies with rare presentations in the oral cavity. Glomus tumors present as a purple or pink vascular nodule or papule, sized <1 cm, and imitate vascular neoplasms such as hemangiopericytoma or hemangioma. Glomus tumors represent less than 2% of all benign soft tissue tumors. Only 27 cases of benign glomus tumors with oral cavity involvement have been reported to date. The most-reported oral tumors involved the lips (54.2%), followed by hard palate, gingiva, tongue, and buccal mucosa. The mean age of presentation of the labial glomus tumors is 48.7 years, with no gender predilection, in contrast to the subungual site, which occurs more in females. The etiology of the glomus tumors remains unknown. Subungual glomus tumors present as stabbing pain, cold intolerance, and tenderness of the fingertips, whereas labial glomus tumors mostly present as a painless, small, and slow-growing lesion. Treatment is surgical resection of the tumor. The recurrence rate of labial glomus tumors is unclear. In this article, we present the case of a 62-year-old man with a 2-month history of painless, soft lump on the mucosal surface of the lower left lip. Excisional resection of the tumor was performed in the clinic, and the histopathologic finding was consistent with solid glomus tumor. At 1 year follow-up there was no recurrence.