Bare lymphocyte syndrome

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Bare Lymphocyte Syndrome

Bare Lymphocyte Syndrome (pronunciation: /bɛər ˈlɪmfəsaɪt ˈsɪndroʊm/) is a rare genetic disorder that affects the immune system.

Etymology

The term "Bare Lymphocyte Syndrome" is derived from the characteristic feature of the disorder where the lymphocytes, a type of white blood cell, appear "bare" or lacking in certain proteins on their surface.

Overview

Bare Lymphocyte Syndrome is characterized by a severe reduction in the number of Major Histocompatibility Complex (MHC) class II molecules on the surface of certain cells. This leads to a decreased ability of the immune system to fight off infections, resulting in recurrent and often severe bacterial, viral, and fungal infections.

Types

There are two types of Bare Lymphocyte Syndrome: Type I and Type II.

  • Bare Lymphocyte Syndrome Type I is characterized by a deficiency in MHC class I molecules. This type is extremely rare and its symptoms and severity can vary widely.
  • Bare Lymphocyte Syndrome Type II is characterized by a deficiency in MHC class II molecules. This type is more common and often results in severe immune deficiency.

Symptoms

Symptoms of Bare Lymphocyte Syndrome can vary widely but often include recurrent infections, chronic diarrhea, failure to thrive, and skin rashes.

Diagnosis

Diagnosis of Bare Lymphocyte Syndrome is often made through genetic testing and analysis of the patient's immune system.

Treatment

Treatment for Bare Lymphocyte Syndrome often involves managing the symptoms and preventing infections. In severe cases, a bone marrow transplant may be considered.

See Also

External links

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