학술논문

Lymphomatoid granulomatosis in a patient with DOCK8 deficiency.
Document Type
Article
Source
Pediatric Allergy & Immunology. Jun2022, Vol. 33 Issue 6, p1-5. 5p.
Subject
*JOB'S syndrome
*BRONCHIECTASIS
*CHOLANGITIS
*DIFFUSE large B-cell lymphomas
*INTRAHEPATIC bile ducts
Language
ISSN
0905-6157
Abstract
Combined immunodeficiency, EBV infection, lymphoproliferative disorder, DOCK8 deficiency, lymphomatoid granulomatosis Keywords: combined immunodeficiency; DOCK8 deficiency; EBV infection; lymphomatoid granulomatosis; lymphoproliferative disorder EN combined immunodeficiency DOCK8 deficiency EBV infection lymphomatoid granulomatosis lymphoproliferative disorder 1 5 5 06/29/22 20220601 NES 220601 To the Editor, Combined immunodeficiency (CID) due to DOCK8 deficiency is an inborn error of immunity (IEI) characterized by defects in T and B lymphocytes, resulting in severe atopic dermatitis, recurrent cutaneous viral infections, susceptibility to the development of neoplasms, and normal/elevated levels of IgE.1 The spectrum of manifestations includes allergy, autoimmunity, neoplasms, and recurrent infections. Furthermore, patients with DOCK8 deficiency are particularly susceptible to viruses belonging to the herpes family.2 Lymphomatoid granulomatosis (LYG) is a lymphoproliferative disorder associated with chronic EBV infection. [Extracted from the article]