An immunodeficiency disorder occurring in boys that is marked by defects in both T and B cell function. Patients have eczema, recurring bloody diarrhea, thrombocytopenia (an abnormal decrease in the number of blood platelets) with bleeding tendency and infections, especially of the ears. The prognosis has improved and some patients treated with spelenectomy have reached adulthood. Patients who have been successfully treated by bone marrow transplantation should have a normal life expectancy.
No programs.