Authors :
Mokhantar Khaoula; Allaoui Abire; Bousfiha Ahmed Aziz; Ouazahrou Kaoutar; Moudatir Mina
Volume/Issue :
Volume 8 - 2023, Issue 2 - February
Google Scholar :
https://bit.ly/3IIfn9N
Scribd :
https://bit.ly/3E1GJYH
DOI :
https://doi.org/10.5281/zenodo.7638585
Abstract :
Common variable immune deficiency (CVID)
is the most common symptomatic immune deficiency in
adults. It is defined by primary hypogammaglobulinemia
with B-cells (CD19) present. It is a heterogeneous
syndrome characterized clinically by recurrent bacterial
infections (an almost constant feature), the frequency of
autoimmune and/or granulomatous manifestations, and
the presence of a lymphoproliferative syndrome
(follicular hyperplasia, lymph nodes…). The seriousness
of infections and their repetition should suggest the
diagnosis, which will be confirmed by electrophoresis of
the proteins and immunoglobulin tests. The treatment is
based on regular substitution of polyvalent
immunoglobulins, using intravenous or subcutaneous
injection. The disease pathophysiology is poorly
understood, but the immunological study of lymphocyte
subpopulations, particularly B lymphocytes, and the
recent description of certain genetic defects have
improved our understanding. This study aims to cite the
latest clinical and immunological characteristics of
patients with CVID, as well as the genetic and physiopathogenic advances concerning this immune deficiency.
In order to propose a new criteria to explore this
pathology conducted to rule out other possible causes of
hypogammaglobulinemia and differentials. Diagnoses
using these criteria will enable patients with CVID to
receive the best possible care.
Keywords :
CVID, Immunodeficiency, Hypogammaglobulinemia, B Cell
Common variable immune deficiency (CVID)
is the most common symptomatic immune deficiency in
adults. It is defined by primary hypogammaglobulinemia
with B-cells (CD19) present. It is a heterogeneous
syndrome characterized clinically by recurrent bacterial
infections (an almost constant feature), the frequency of
autoimmune and/or granulomatous manifestations, and
the presence of a lymphoproliferative syndrome
(follicular hyperplasia, lymph nodes…). The seriousness
of infections and their repetition should suggest the
diagnosis, which will be confirmed by electrophoresis of
the proteins and immunoglobulin tests. The treatment is
based on regular substitution of polyvalent
immunoglobulins, using intravenous or subcutaneous
injection. The disease pathophysiology is poorly
understood, but the immunological study of lymphocyte
subpopulations, particularly B lymphocytes, and the
recent description of certain genetic defects have
improved our understanding. This study aims to cite the
latest clinical and immunological characteristics of
patients with CVID, as well as the genetic and physiopathogenic advances concerning this immune deficiency.
In order to propose a new criteria to explore this
pathology conducted to rule out other possible causes of
hypogammaglobulinemia and differentials. Diagnoses
using these criteria will enable patients with CVID to
receive the best possible care.
Keywords :
CVID, Immunodeficiency, Hypogammaglobulinemia, B Cell