immunodeficiency

[im″u-no-de-fish´en-se]

a deficiency of immune response or a disorder characterized by deficient immune response; classified as antibody (B cell), cellular (T cell), or combined deficiency disorders. Antibody immunodeficiencies are marked by hypo- or dysgammaglobulinemia, recurrent bacterial otitis media, and sinopulmonary infections. Cellular immunodeficiencies are characterized by recurrent low-grade or opportunistic infections, by graft-versus-host disease or reaction after blood transfusions, and by severe disease after immunization with live vaccines. See also acquired immunodeficiency syndrome.

common variable immunodeficiency (CVID) a heterogeneous group of disorders characterized by hypogammaglobulinemia, decreased antibody production in response to antigenic challenge, and recurrent pyogenic infections, often associated with hematologic and autoimmune disorders. Most patients have normal numbers of circulating B cells but lack plasma cells and appear to have an intrinsic defect of B cell differentiation. However, two other forms are also recognized: that due to a disorder of T lymphocyte regulation and that due to production of autoantibodies against T and B lymphocytes.

severe combined immunodeficiency (SCID) any of several rare congenital diseases, some of autosomal recessive and some of X-linked inheritance, in which both humoral and cell-mediated immunity fail to develop normally and T lymphocytes are absent or nearly so. In some forms, B lymphocytes are also absent. Early diagnosis is essential to prevent opportunistic infections. Persistent diarrhea, chronic mucocutaneous candidiasis, and failure to thrive may occur in infancy. Blood transfusions can result in graft-versus-host disease and routine vaccinations in fatal infection. Unless immune function is restored by a matched-donor bone marrow or fetal tissue transplantation or the patient is kept in complete isolation, the prognosis is poor.

CVID

Common variable immune deficiency, see there.

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References in periodicals archive

Gastrointestinal manifestations occur in about 10% to 20% of patients with CVID, and may be the initial or sole presentation.

Gastrointestinal Presentations of Common Variable Immunodeficiency: Hiding in Plain Sight

CVID is a heterogeneous primary immunodeficiency and the search for causative or susceptibility gene(s) has been progressing for the last two decades.

TNFRSF13B/TACI Alterations in Turkish Patients with Common Variable Immunodeficiency and IgA Deficiency

Iatrogenic hypogammaglobulinemia is an acquired hypogammaglobulinemia caused by various drugs including antirheumatic and antiepileptic medications, but clinically, it may be difficult to distinguish it from CVID. In contrast to this, common variable immune deficiency is a congenital disorder that involves low levels of most or all of immunoglobulins, lack of B-lymphocytes or plasma cells that are capable of producing antibodies, and repeated bacterial infections [11].

Hypogammaglobulinemia Observed One Year after Rituximab Treatment for Idiopathic Thrombocytopenic Purpura

CVID is characterized by low levels of most or all of the immunoglobulin classes, as well as a lack of B lymphocytes or plasma cells that are capable of producing antibodies.

Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency

Three patients had CVID, but none of them has excreted enteroviruses so far, as reported in other studies (14,31).

Poliovirus Excretion in Children with Primary Immunodeficiency Disorders, India

CVID is the most frequent symptomatic primary immunodeficiency.

Secondary Amenorrhea in a Patient with Common Variable Immunodeficiency

There were no radiological or pathological features suggestive of bronchiectasis or granulomatous-lymphocytic interstitial lung disease (GLILD), which are typical pulmonary complications of CVID [35].

TINF2 Gene Mutation in a Patient with Pulmonary Fibrosis

However, no patient with URTI alone was found among XLA and CVID patients and the diagnosis was made with a history of serious infection including pneumonia, sepsis, osteomyelitis accompanying the history of URTI in these patients.

Profile of the patients who present to immunology outpatient clinics because of frequent infections

Low levels of immunoglobulins with normal B lymphocyte numbers suggest CVID. High IgM levels with low IgG/IgA/IgE occur in class switch recombination deficiencies.

Primary immunodeficiency diseases: 'look deeper into nature, and then you will understand everything better.' - Albert Einstein

I was also diagnosed with an immune disease called Common Variable Immunodeficiency (CVID).

Keri Pike