Primary cutaneous peripheral T-cell lymphoma in a patient with X-linked agammaglobulinaemia.

摘要

Madam, Primary cutaneous peripheral T-cell lymphoma is a rare disease constituting approximately 10% of all cutaneous T-cell lymphomas. X-linked agammaglobulinaemia (XLA), characterized by a profound deficiency of B cells, is caused by mutations of the gene encoding the cytoplasmic Bruton tyro-sine kinase (BTK).Gompels et al. have undertaken a retrospective study of antibody-deficient patients with and without lymphoma. They assessed tissue from 34 patients with antibody deficiency presenting during the past 20 years, of whom 19 patients had progressed to lymphoma and 15 had not. Of those patients with a lymphoma, 16 had a diagnosis of common variable immunodeficiency, two subclass deficiency and one XLA. The authors confirmed that lymphoma in antibody deficiency is predominantly B cell in origin.