Open AccessDiminished CD40L expression on T-cells in a case of disseminated cryptococcosis
Author(s) -
Supreet Kumar Mohanty,
Deepshi Thakral,
Devika Gupta,
Prabin Kumar,
Dipendra Kumar Mitra
Publication year - 2018
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/ijpm.ijpm_761_16
Subject(s) - cd154 , cd40 , immunology , immunodeficiency , immunoglobulin e , antibody , medicine , biology , immune system , in vitro , cytotoxic t cell , biochemistry
X-linked hyperimmunoglobulin M (HIGM) syndrome may increase the susceptibility of patients to disseminated cryptococcal infections primarily due to CD40L deficiency that causes defective cross talk between T- and B-cells, thus preventing class switching. In HIGM syndrome, serum IgM levels are elevated with severe reduction in serum immunoglobulin G (IgG) and IgA levels. In addition, the expression of CD40L (CD154) on in vitro-activated T-cells is severely reduced or absent. Here, we describe a rare, and perhaps, the first reported case in India of a 3-year-old male child with X-linked HIGM immunodeficiency syndrome who developed disseminated Cryptococcosis. Evaluation of the serum IgG profile of the patient revealed increased serum IgM levels with reduced IgG and IgA levels. Both the frequency and the function of T-cells, primarily CD40L on activated T-cells, showed weak expression suggestive of HIGM syndrome.