Thymoma associated with hypogammaglobulinaemia and pure red cell aplasia

نویسندگان

  • Juan Briones
  • Mirentxu Iruretagoyena
  • Héctor Galindo
  • Claudia Ortega
  • Pablo Zoroquiain
  • José Valbuena
  • Francisco Acevedo
  • Mauricio Ocqueteau
  • Cesar Sánchez
چکیده

Thymomas are neoplasias that begin in the thymus and develop in the anterior mediastinum. They are commonly associated with a variety of systemic and autoimmune disorders, such as pure red cell aplasia, hypogammaglobulinaemia, pancytopaenia, collagen diseases, and, most commonly, myasthenia gravis. The presence of inter-current infections, especially diarrhoea and pneumonia, in the presence of lymphocyte B depletion and hypogammaglobulinaemia is known as Good's syndrome and may affect up to 5% of patients with thymoma. While anaemia is present in 50%-86% of patients with Good's syndrome, only 41.9% of cases present pure red cell aplasia. Concomitance of these two conditions has only been rarely studied. We report on the case of a 55-year-old man diagnosed with advanced thymoma, who, during the progression of his disease, developed signs and symptoms suggesting Good's syndrome and pure red cell aplasia. We also performed a brief review of the literature concerning this association, its clinical characteristics, and treatment.

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Leukemia and myelodysplasia in patients with essential thrombocythemia treated with cytotoxic agents.

1. Good RA. Agammaglobulinemia: a provocative experiment of nature. Bull Univ Minn Hosp Min Med Found 1954; 26:1-19. 2. Levinson AI, Hoxie JA, Kornstein MJ, Zembryki D, Matthews DM, Schreiber AD. Absence of the OKT4 epitope on blood T cells and thymus cells in a patient with thymoma, hypogammaglobulinemia, and red blood cell aplasia. J Allergy Clin Immunol 1985; 76: 433-9. 3. Oshimi K. Granular...

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عنوان ژورنال:

دوره 7  شماره 

صفحات  -

تاریخ انتشار 2013