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"Borderline" idiopathic CD4+ T-cell lymphocytopenia presenting with atypical progressive multifocal leukoencephalopathy. 2020

Clemente Dato, and Andrea Elefante, and Cinzia Coppola, and Mariarosa Anna Beatrice Melone, and Giacomo Lus, and Antonella Costagliola, and Giorgia Bruno, and Gianfranco Puoti
Department of Advanced Medical and Surgical Sciences, Second Division of Neurology - University of Campania "Luigi Vanvitelli", via Pansini 5, 80131 Naples, Italy.

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.

UI MeSH Term Description Entries
D007968 Leukoencephalopathy, Progressive Multifocal An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7) Encephalitis, JC Polyomavirus,Progressive Multifocal Leukoencephalopathy,JC Polyomavirus Encephalopathy,Encephalopathies, JC Polyomavirus,Encephalopathy, JC Polyomavirus,JC Polyomavirus Encephalitis,Leukoencephalopathies, Progressive Multifocal,Multifocal Leukoencephalopathies, Progressive,Multifocal Leukoencephalopathy, Progressive,Progressive Multifocal Leukoencephalopathies
D008297 Male Males
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D015496 CD4-Positive T-Lymphocytes A critical subpopulation of T-lymphocytes involved in the induction of most immunological functions. The HIV virus has selective tropism for the T4 cell which expresses the CD4 phenotypic marker, a receptor for HIV. In fact, the key element in the profound immunosuppression seen in HIV infection is the depletion of this subset of T-lymphocytes. T4 Cells,T4 Lymphocytes,CD4-Positive Lymphocytes,CD4 Positive T Lymphocytes,CD4-Positive Lymphocyte,CD4-Positive T-Lymphocyte,Lymphocyte, CD4-Positive,Lymphocytes, CD4-Positive,T-Lymphocyte, CD4-Positive,T-Lymphocytes, CD4-Positive,T4 Cell,T4 Lymphocyte
D018344 T-Lymphocytopenia, Idiopathic CD4-Positive Reproducible depletion of CD4+ lymphocytes below 300 per cubic millimeter in the absence of HIV infection or other known causes of immunodeficiency. This is a rare, heterogeneous syndrome and does not appear to be caused by a transmissible agent. CD4-Positive T-Lymphocytopenia, Idiopathic,Idiopathic CD4-Positive T-Lymphocytopenia,CD4+ T-Lymphocytopenia,CD4+ T-Lymphocytopenia, Idiopathic,Idiopathic CD4 Positive T-Lymphocytopenia,Idiopathic CD4+ T-Lymphocytopenia,T-Lymphocytopenia, Idiopathic CD4+,CD4 Positive T Lymphocytopenia, Idiopathic,CD4+ T Lymphocytopenia,CD4+ T Lymphocytopenia, Idiopathic,CD4+ T-Lymphocytopenias,Idiopathic CD4+ T Lymphocytopenia,T Lymphocytopenia, Idiopathic CD4 Positive,T Lymphocytopenia, Idiopathic CD4+,T-Lymphocytopenia, CD4+,T-Lymphocytopenias, CD4+

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