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Overlap of lymphatic dysplasia in Fontan-associated protein-losing enteropathy and Mucosa-Associated Lymphoid Tissue (MALT lymphoma): implications for management of protein-losing enteropathy

Published online by Cambridge University Press:  05 October 2020

Joshua K. Meisner Open the ORCID record for Joshua K. Meisner [Opens in a new window] ,
Sarah Koblick ,
Emily B. Walling ,
Raja Rabah  and
Kurt R. Schumacher
Joshua K. Meisner*
Affiliation:
Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA48109
Sarah Koblick
Affiliation:
Division of Pediatric Hematology and Oncology, University of Michigan, Ann Arbor, MI, USA48109
Emily B. Walling
Affiliation:
Division of Pediatric Hematology and Oncology, University of Michigan, Ann Arbor, MI, USA48109
Raja Rabah
Affiliation:
Division of Pediatric and Perinatal Pathology, University of Michigan, Ann Arbor, MI, USA48109
Kurt R. Schumacher
Affiliation:
Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA48109
*
Author for correspondence: Joshua K. Meisner, C.S. Mott Children’s Hospital, Congenital Heart Center, Division of Pediatric Cardiology, 1540 E. Hospital Drive, SPC 4204, Ann Arbor, Michigan48109-4204, USA. Tel: +734-764-5177; Fax: +734-936-9470. E-mail: [email protected]
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Abstract

Lymphatic vessel dysplasia is associated with Fontan-associated protein-losing enteropathy. Extra nodal non-Hodgkin lymphomas including mucosa-associated lymphoid tissue (MALT lymphoma) are associated with lymphatic vessel dysplasia. Here, we describe the case of a 7-year-old with Fontan-associated protein-losing enteropathy who developed MALT lymphoma with a clinical course indicative of interaction between these pathologies and improvement in protein-losing enteropathy after MALT lymphoma treatment. This case suggests a pathophysiologic overlap which has implications for the management of Fontan-associated protein-losing enteropathy.

Keywords

MALT lymphomaprotein-losing enteropathyintestinal lymphangiectasiacavopulmonary anastomosis
Type
Brief Report
Information
Cardiology in the Young , Volume 30 , Issue 12 , December 2020 , pp. 1973 - 1975
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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