D2.250 - Can drug allergy develop in x-linked agammaglobulinemia (xla)? case report

X-linked Agammaglobulinemia (XLA) is a rare genetic disorder of B-lymphocyte differentiation, characterized by the absence or paucity of circulating B cells, markedly reduced levels of all serum immunoglobulin isotypes, lack of specific antibody production. Bruton Tyrosine Kinase (BTK) gene mutation response from disease. Allergy can also occur in XLA patients. Both IgE mediated and non-IgE mediated symptoms have been reported. However, drug allergy has not been reported to date. In this report, we present a unique case of a young patient affected with XLA who suffered from urticaria that developed after antibiotic therapy.

Case: A 16-year-old male patient was referred to our Immunology clinic when he was 5 years old due to frequent infections and his serum immunoglobulin G, M and A values were low. In his medical history, it was learned that he had moderate-severe respiratory tract infections since he was 1 year old. His aunt's son was also receiving immunoglobulin replacement (IGRT) treatment with a diagnosis of immune deficiency. In his physical examination, tonsils, lymph node or hepatosplenomegaly were not determined. XLA diagnosed with immunologic evaluation and in the BTK gene, p.Arg 255X hemizygous nonsense mutation was detected. Chronic sinusitis and bronchiectasis developed during the follow-up. When he admitted to our clinic with fever and cough, he hospitalized because of pneumonia, pseudomonas aeroginosa was grown in the sputum. Piperacillin tazobactam therapy was started because of the bacteria is sensitive. Urticaria developed all over the body 15 minutes after the drug was administered. It regressed with antihistaminc drugs. No spesific labarotary findings were determined for urticeria except eosinophilia (6%-450/mm3). After the second dose of piperacillin tazobactam, urticaria developed again. The antibiotic therapy was changed and urticaria didn’t develop. Skin prick test, penicillin specific IgE test were negative. Drug skin test was planned. The patient's follow-up continues in this manner.

While BTK inhibitors are currently being developed for the treatment of many diseases, especially allergy, the development of drug allergy in XLA suggests that we need to examine BTK physiology in more detail.