GOOD'S SYNDROME ASSOCIATED WITH CYTOMEGALOVIRUS PNEUMONIA : CASE REPORT AND LITERATURE REVIEW.

Good's syndrome is a rare entity defined by the association of a thymoma with an acquired immune deficiency characterized by hypogammaglobulinemia. We report a case of a 62 year old patient, Diagnosed and treated for thymoma. The patient presented 4 months later with severe pneumonia. Biological workup revealed hypogammaglobulinemia at the expense of all immunoglobulin classes, lymphocytopenia involving B and T CD4+ lymphocytes leading to the diagnosis of Good's syndrome. Cytomegalovirus DNA testing came back positive. The patient received immunoglobulin infusion, noting an improvement in his general condition. Good's syndrome represents 6 to 11% of para-thymic syndromes. Its pathogenesis remains unknown. Its clinical presentation is diverse, with either a mediastinal syndrome or recurrent severe infections. Cytomegalovirus is found in 40% of cases. Management is based on thymomectomy and introduction of anti-infectious agents. Hypogammaglobulinemia, rarely improved by thymomectomy, requires the addition of systemic immunoglobulins. The rarity, clinical diversity, and severity of Good's Syndrome justifies rigorous clinical and immunologic investigation when diagnosing thymoma or the occurrence of repeated severe infections, especially since therapeutic management, despite the absence of a well-defined consensus, can improve the prognosis and survival of patients.