DOCK8 deficiency is a combined immunodeficiency disease that is characterized by severe problems with recurrent, persistent, and progressive infections, especially of the skin. This is a major contributing factor to the high morbidity and mortality seen in this disease. However, another key feature of DOCK8 deficiency is the allergic disease: patients typically have severe eczematous dermatitis, asthma, and life-threatening food allergies, often accompanied by high levels of serum IgE and eosinophilia. Most studies to date have focused on the infectious but not the allergic complications of this disease. In FY2016, we studied a cohort of twelve DOCK8-deficient patients who had undergone hematopoietic stem cell transplantation at the NIH Clinical Center. Although such treatment cures the eczema and infectious complications of DOCK8 deficiency, we found that the food allergies were not cured in significant number of patients. This study provides important information regarding the timing of transplantation and need for continued medical management after transplantation in DOCK8-deficient patients. Furthermore, as the first systemic study of hematopoietic stem cell transplantation for food allergies, the results are also more generalizable to other food allergic individuals who do not have DOCK8 deficiency.
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