Good's syndrome and pure white cell aplasia complicated by cryptococcus infection: A case report and review of the literature

J Clin Immunol. 2014 Apr;34(3):283-8. doi: 10.1007/s10875-014-0014-7. Epub 2014 Mar 14.

Abstract

Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good's syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good's syndrome. Lethal opportunistic infections are a feature of Good's syndrome, usually occurring in those patients with associated severe T lymphocyte defects. Although the cryptococcus is a recognised fungal pathogen in patients with other causes of CD4+ T cell lymphopenia, surprisingly this complication has not been reported in patients with Good's syndrome. We now describe a 70 year old man with Good's syndrome and pure white cell aplasia who presented with disseminated cryptococcosis, and provide an up-to-date review of the relevant literature. Despite meningeal involvement our patient recovered after combined treatment with intravenous globulin, granulocyte stimulating growth, corticosteroids and antifungal therapy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Biopsy
  • Bone Marrow / pathology
  • Cryptococcosis / complications*
  • Cryptococcosis / diagnosis
  • Cryptococcosis / drug therapy
  • Female
  • Humans
  • Leukopenia / diagnosis
  • Leukopenia / etiology*
  • Thymoma / complications*
  • Thymoma / diagnosis
  • Thymoma / surgery
  • Thymus Neoplasms / complications*
  • Thymus Neoplasms / diagnosis
  • Thymus Neoplasms / surgery