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BackgroundHyper IgE syndrome is a rare heterogeneous immunodeficiency syndrome which is characterized by recurrent episodes of cutaneous and respiratory tract infections and development of cold abscesses. This syndrome is also associated with malignancies, most notability hematologic malignancies.Case presentationIn this case report, we discuss a 27-year-old male with proven hyper IgE syndrome and effected by tuberculosis, who developed an anaplastic large cell lymphoma, a very rare and aggressive subtype of lymphoma. We will discuss imaging findings in this rare case. The case presented here had a large mediastinal mass, which had encircled the aorta, and was accompanied by subcarinal lymphadenopathy and inguinal lymphadenopathy.ConclusionsSystemic anaplastic large cell lymphoma is a rare subtype of lymphoma which is rarely associated with hyper IgE syndrome. In this case, both lymphoma and tuberculosis infection were witnessed in the same patient, showing a classic example of immune dysregulation.

Systemic anaplastic large cell lymphoma presenting as a huge mediastinal mass in a case of hyper IgE syndrome: a case report