Leukoencephalopathy- arthritis- colitis- and hypogammaglobulinema
Overview
A rare syndrome characterized by brain disease, arthritis, colitis and immune system problems.
Symptoms
* Leukoencephalopathy * Arthritis * Colitis * Low blood gamma globulin levels - IgG1-subclass * Low B cell levels
Causes
1.Certain viral infectious have been shown to cause transient or permanent immune deficiency. 2.Malignancies such as leukemias, multiple myeloma, and neuroblastoma may also manifest hypogammaglobulinemia. 3.Excessive protein loss from the GI tract may result in hypogammaglobulinemia; however, primary antibody deficiency may also cause chronic diarrhea. Therefore, any protein-losing enteropathy should be considered in patients presenting with hypogammaglobulinemia. In these situations, specific antibody responses are intact, and circulating B cells are normal. On the other hand, GI protein loss may also occur from lymphatic obstruction in diseases such as intestinal lymphangiectasia. Concomitant loss of lymphocytes into the intestinal tract may result in lymphopenia. 4. Finally, cow's milk allergy may also result in hypogammaglobulinemia, possibly due to immunoglobulin leakage through inflamed GI mucosa.30 Avoidance of the allergen resulted in normalization of immunoglobulin levels