Combined Immunodeficiency Disorders
This is characterized by severe T cells and B cells deficiency with intact antibody response.
This occurs sporadically and is transmitted as X-linked and autosomal recessive traits.
Clinically, this is found in infants who usually die within 2 years of life. They usually present as:- failure to thrive
– chronic otitis media
– chronic diarrhea
– oropharyngeal candidiasis
– and recurrent pneumonia.
This profound system immunodeficiency disorder may occur alone or in combination with other abnormalities or diseases. It has three types and are as follows:
* Type 1 is SCID with short- limbed dwarfism
* Type 2 is isolated cellular immunodeficiency with cartilage hair hypoplasia
* Type 3 is antibody deficiency
Severe combined immunodeficiency disorder or SCID may be associated with adenosine deaminase deficiency and Letterer- Siwe’s disease and acrodermatitis enteropathica.
The treatment of SCID is bone marrow transplantation.