21 January, 2022
A Unique case of Hyper-IgE Syndrome

A four-year-old girl with the hyper-IgE syndrome (a kind of primary immunodeficiency) presented to our hospital with extensive skin excoriation with bleeding and a history of recurrent infections. Our team of pediatric hemato-oncologists planned and decided on BMT as the curative treatment.

Though there was no full match found in the family, the elder sibling (sister) was a 50% match and hence haploidentical BMT was performed in April 2021. Now, after more than 260 days ( >8months) the child is fine with no skin lesions and infections and has 100% donor cells. All the skin lesions have completely disappeared, no infections. This is a rare condition with an incidence of 1 over 3.5 lac population.

Currently, there is only one published case of Hyper IgE syndrome with DOCK8 mutation, from India, where the patient could not survive even after receiving the treatment.

These are rare primary immunodeficiency conditions without stem cell transplantation; their outcome would be dismal and their life span shortened; the only curative treatment is BMT.

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