Pulmonary Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Associated with Granulomatous Inflammation in a Child with Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome)

Patients with immunodeficiency disorders have an increased incidence of lymphoproliferative disorders; however, only 4 such patients with DiGeorge/chromosome 22q11.2 deletion syndrome have been reported. We report a case of a pulmonary Epstein-Barr virus–negative extranodal marginal zone lymphoma of...

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Bibliographic Details
Published in:The Journal of pediatrics 2012-11, Vol.161 (5), p.954-958.e1
Main Authors: Pongpruttipan, Tawatchai, MD, Cook, James R., MD, PhD, Reyes-Mugica, Miguel, MD, Spahr, Jonathan E., MD, Swerdlow, Steven H., MD
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
case studies
children
chromosomes
Chromosomes, Human, Pair 22 - genetics
Comorbidity
DiGeorge Syndrome - complications
DiGeorge Syndrome - diagnosis
DiGeorge Syndrome - therapy
Female
Gene Deletion
General aspects
Granuloma - metabolism
Hematologic and hematopoietic diseases
Humans
immunosuppression (physiological)
In Situ Hybridization, Fluorescence
inflammation
Inflammation - metabolism
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
lymphoma
Lymphoma, B-Cell, Marginal Zone - complications
Lymphoma, B-Cell, Marginal Zone - diagnosis
Lymphoma, B-Cell, Marginal Zone - therapy
Medical sciences
Multiple Pulmonary Nodules - complications
Multiple Pulmonary Nodules - diagnosis
Multiple Pulmonary Nodules - therapy
patients
Pediatrics
Tomography, X-Ray Computed - methods
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Description
Summary:Patients with immunodeficiency disorders have an increased incidence of lymphoproliferative disorders; however, only 4 such patients with DiGeorge/chromosome 22q11.2 deletion syndrome have been reported. We report a case of a pulmonary Epstein-Barr virus–negative extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a child with this syndrome.
ISSN:0022-3476
1097-6833
DOI:10.1016/j.jpeds.2012.07.003