"Borderline" idiopathic CD4+ T-cell lymphocytopenia presenting with atypical progressive multifocal leukoencephalopathy

J Neuroimmunol. 2020 Dec 15:349:577420. doi: 10.1016/j.jneuroim.2020.577420. Epub 2020 Sep 29.

Abstract

Idiopathic CD4+ lymphocytopenia (ICL) is a rare disorder characterized by low counts of CD4+ cells (<300/mm3) in absence of other known causes of immunosuppression. A few cases of progressive multifocal leukoencephalopathy (PML) were reported in association with ICL with variable outcome. We describe the case of a 40 year-old man diagnosed with PML, which showed a monophasic course. Causes of primary and secondary immunodeficiency were ruled out, only a "borderline" ICL was found. This case highlights that a severe immunodepression could not be an absolute prerequisite in developing PML and also points the attention on current definition of ICL.

Keywords: ICL; Idiopathic CD4(+) T cell lymphocytopenia; JCV; John Cunningham vvirus; PML; Progressive multifocal leukoencephalopathy.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • CD4-Positive T-Lymphocytes / metabolism
  • Diagnosis, Differential
  • Humans
  • Leukoencephalopathy, Progressive Multifocal / blood*
  • Leukoencephalopathy, Progressive Multifocal / diagnostic imaging*
  • Male
  • T-Lymphocytopenia, Idiopathic CD4-Positive / blood*
  • T-Lymphocytopenia, Idiopathic CD4-Positive / diagnostic imaging*