Good syndrome (GS) is a rare adult-onset immunodeficiency disease characterised by hypogammaglobulinaemia and thymoma. Here we describe a 72-year-old male patient who was diagnosed with GS when he was 62, after a two-year history of recurrent respiratory infections. A chest CT scan showed a mediastinal mass which was surgically removed; its histology revealed a thymoma. The patient was hypogammaglobulinaemic and his clinical condition dramatically improved after starting an appropriate dosage of IVIG. Two years ago he developed a normochromic normocytic anaemia requiring several transfusions. A bone marrow biopsy revealed a myelodysplastic syndrome. The patient started cyclosporine and the anaemia gradually improved, achieving transfusion independence.
Myelodysplasia and Good syndrome. A case report / Di Renzo, M; Pasqui, A L; Voltolini, L; Gotti, G; Pompella, G; Auteri, A. - In: CLINICAL AND EXPERIMENTAL MEDICINE. - ISSN 1591-8890. - ELETTRONICO. - 8:(2008), pp. 171-173. [10.1007/s10238-008-0175-x]
Myelodysplasia and Good syndrome. A case report
Voltolini, L;Gotti, G;
2008
Abstract
Good syndrome (GS) is a rare adult-onset immunodeficiency disease characterised by hypogammaglobulinaemia and thymoma. Here we describe a 72-year-old male patient who was diagnosed with GS when he was 62, after a two-year history of recurrent respiratory infections. A chest CT scan showed a mediastinal mass which was surgically removed; its histology revealed a thymoma. The patient was hypogammaglobulinaemic and his clinical condition dramatically improved after starting an appropriate dosage of IVIG. Two years ago he developed a normochromic normocytic anaemia requiring several transfusions. A bone marrow biopsy revealed a myelodysplastic syndrome. The patient started cyclosporine and the anaemia gradually improved, achieving transfusion independence.I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.