Abstract
Primary immunodeficiencies (PIDs) represent a heterogeneous group of congenital disorders, often associated with specific genetic mutations. They are characterized by a varied spectrum of clinical manifestations, malignant tumors being the second cause of death among patients with primary immunodeficiencies after infections. The article presents the clinical case of a 13-year-old patient with a history of severe ataxia, telangiectasias, frequent respiratory infections, and cervical and submandibular lymphadenopathy diagnosed with IDP Ataxia Telangiectasia based on ESID criteria. Against the background of IDP, he is also diagnosed with Non-Hodgkin’s lymphoma.
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