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Metachronous T‐Lymphoblastic Lymphoma and Burkitt Lymphoma in a Child With Constitutional Mismatch Repair Deficiency Syndrome
Author(s) -
Alexander Thomas B.,
McGee Rose B.,
Kaye Erica C.,
McCarville Mary Beth,
Choi John K.,
Cavender Cary P.,
Nichols Kim E.,
Sandlund John T.
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25989
Subject(s) - medicine , lymphoblastic lymphoma , lymphoma , lynch syndrome , pediatrics , blood cancer , colorectal cancer , cancer , dna mismatch repair , immunology , immune system , t cell
Constitutional mismatch repair deficiency (CMMRD) is a cancer predisposition syndrome associated with a high risk of developing early‐onset malignancies of the blood, brain, and intestinal tract. We present the case of a patient with T‐lymphoblastic lymphoma at the age of 3 years, followed by Burkitt lymphoma 10 years later. This patient also exhibited numerous nonmalignant findings including café au lait spots, lipomas, bilateral renal nodules, a nonossifying fibroma, multiple colonic adenomas, and a rapidly enlarging pilomatrixoma. The spectrum of malignant and nonmalignant neoplasms in this patient highlights the remarkable diversity, and early onset, of lesions seen in children with CMMRD.