Susceptibility to Cryptococcal Meningoencephalitis Associated With Idiopathic CD4+ Lymphopenia and Secondary Germline or Acquired Defects

نویسندگان

  • Anil A. Panackal
  • Lindsey B. Rosen
  • Gulbu Uzel
  • Michael J. Davis
  • Guowu Hu
  • Adebowale Adeyemo
  • Fasil Tekola-Ayele
  • Andrea Lisco
  • Christopher Diachok
  • Jonathan D. Kim
  • Dawn Shaw
  • Irini Sereti
  • Jennifer Stoddard
  • Julie Niemela
  • Sergio D. Rosenzweig
  • John E. Bennett
  • Peter R. Williamson
چکیده

Idiopathic CD4+ lymphopenia (ICL) predisposes to opportunistic infections (OIs) but can often remain asymptomatic and does not have a strong association with monogenic mutations. Likewise, cryptococcal meningoencephalitis, the most common OI in ICL, is not strongly associated with monogenic mutations. In this study, we describe 2 patients with ICL plus an additional immune defect: one from an E57K genetic mutation in the nuclear factor-κβ essential modulator, and the other with acquired autoantibodies to granulocyte-macrophage colony-stimulating factor. Thus, these cases may exemplify a "multi-hit model" in patients with ICL who acquire OIs.

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عنوان ژورنال:

دوره 4  شماره 

صفحات  -

تاریخ انتشار 2017