Disorders involving the immune mechanism
Deficiency of humoral immunity
Hypogammaglobulinemia unspecified
- condition characterized by abnormally low levels of all classes of gamma globulins in the blood.
Selective iga immunodeficiency
Selective igm immunodeficiency
Other selective immunoglobulin deficiencies
Congenital hypogammaglobulinemia
- X-linked agammaglobulinemia. An immunodeficiency state characterized (usually) by profoundly low concentrations of serum immunoglobulins of all classes, although occasionally significant amounts of one or more isotypes can be found. The fundamental defect in XLA affects early lineage B cells.
Immunodeficiency with increased igm
Common variable immunodeficiency
Other deficiency of humoral immunity
Deficiency of cell-mediated immunity
Immunodeficiency with predominant t-cell defect unspecified
Digeorge's syndrome
- congenital syndrome with the absence of the thymus and parathyroids causing impairment of cellular immunity; immunoglobulin levels are normal.
- A congenital anomaly characterized by immunodeficiency, abnormal facies, congenital heart disease, hypocalcemia, and increased susceptibility to infections. Pathologic characteristics include conotruncal abnormalities and absence or hypoplasia of thymus and parathyroid glands. DiGeorge syndrome is associated with abnormalities of chromosome 22. Also known as DiGeorge anomaly. --2004
- Branchial arch defects with craniofacial abnormalities, hearing loss, short stature, and learning difficulty.
- A developmental defect of derivatives of the third and fourth pharyngeal pouches, almost always associated with agenesis or hypoplasia of the thymus and parathyroid gland, characteristic facies with downslanting palpebral fissures and ocular and nasal anomalies, hypocalcemia, cardiovascular anomalies, immunodeficiency, and other variable abnormalities. Patients who survive infancy are usually mentally retarded. DiGeorge syndrome is considered by some researchers as a developmental field defect consisting of several casually distinct disorders, rather than a distinct syndromic entity. Conditions associated with the development of DiGeorge syndrome include diabetic embryopathy, fetal alcohol syndrome, and Zellweger syndrome. Major features of this syndrome have been designated by the Newcastle Upon Tyne Group CATCH 22 (Cardiac, Abnormal facies, Thymic hypoplasia, Cleft palate, and Hypocalcemia), the number 22 indicating deletion of the long arm of chromosome 22 (22q11).
Wiskott-aldrich syndrome
- rare x-linked immunodeficiency syndrome of young boys characterized by eczema, thrombocytopenic purpura and recurrent pyogenic infection; IGM levels are low and IGA and IGE levels are elevated; lymphoreticular malignancies are common.
- A rare, X-linked immunodeficiency syndrome characterized by eczema, thrombocytopenia, and recurrent pyogenic infection. It is seen exclusively in young boys. Typically, IgM levels are low and IgA and IgE levels are elevated. Lymphoreticular malignancies are common.
Nezelof's syndrome
Other deficiency of cell-mediated immunity
Combined immunity deficiency
- group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels; it is inherited as an X-linked or autosomal recessive defect; about half of the patients with autosomal recessive SCID are deficient in the enzyme adenosine deaminase.
- X-linked or autosomal recessive disorder characterized by defects of both humoral and cell mediated immunity, resulting in low or absent antibody levels, leukopenia, marked susceptibility to infections, and early death.--2004
Unspecified immunity deficiency
- deficiency of immune response or a disorder characterized by deficient immune response; classified as antibody (B cell), cellular (T cell), or combined immunodeficiency, or phagocytic dysfunction disorders.
- The inability of the body to produce an immune response.
- The decreased ability of the body to fight infection and disease.
- A deficiency of immune response or a disorder characterized by deficient immune response.
- Syndromes in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral.
Autoimmune disease not elsewhere classified
- heading term for a diverse group of disorders characterized by pathologenic immune reactions to autologous (self) tissue; for nonnpatholgical, developmental, or phenomenological aspects of autoimmunity, use AUTOIMMUNITY.
- A condition in which the body recognizes its own tissues as foreign and directs an immune response against them.
- A disorder resulting from loss of function or tissue destruction of an organ or multiple organs, arising from humoral or cellular immune responses of the individual to his own tissue constituents. It may be systemic (e.g., systemic lupus erythematosus), or organ specific, (e.g., thyroiditis). -- 2003
Other specified disorders involving the immune mechanism
Unspecified disorder of immune mechanism
