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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: jmeisner@med.umich.edu
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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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References

Claviez, A, Meyer, U, Dominick, C, et al. MALT lymphoma in children: a report from the NHL-BFM study group. Pediatr Blood Cancer 2006; 47: 210–214.CrossRefGoogle ScholarPubMed
Grulich, AE, Vajdic, CM, Cozen, W. Altered immunity as a risk factor for Non-Hodgkin lymphoma. Cancer Epidemiol Biomarkers Prev 2007; 16: 405–408.CrossRefGoogle ScholarPubMed
Sokol, H, Beaugerie, L, Maynadié, M, et al. Excess primary intestinal lymphoproliferative disorders in patients with inflammatory bowel disease. Inflamm Bowel Dis 2012; 18: 2063–2071.CrossRefGoogle ScholarPubMed
Laharie, D, Degenne, V, Laharie, H, et al. Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. Eur J Gastroenterol Hepatol 2005; 17: 1417–1419.CrossRefGoogle Scholar
Pratz, KW, Dingli, D, Smyrk, TC, Lust, JA. Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore) 2007; 86: 210–214.CrossRefGoogle ScholarPubMed
Umar, SB, DiBaise, JK. Protein-losing enteropathy: case illustrations and clinical review. Am J Gastroenterol 2010; 105: 43–49; quiz 50.CrossRefGoogle ScholarPubMed
Itkin, M, Piccoli, DA, Nadolski, G, et al. Protein-losing enteropathy in patients with Congenital Heart Disease. J Am Coll Cardiol 2017; 69: 2929–2937.CrossRefGoogle ScholarPubMed
Farinha, P, Gascoyne, RD. Molecular pathogenesis of Mucosa-Associated Lymphoid Tissue lymphoma. J Clin Oncol 2005; 23: 6370–6378.CrossRefGoogle ScholarPubMed
Schumacher, KR, Cools, M, Goldstein, BH, et al. Oral Budesonide treatment for protein-losing enteropathy in Fontan-Palliated patients. Pediatr Cardiol 2011; 32: 966–971.CrossRefGoogle ScholarPubMed
Riaz, IB, Faridi, W, Patnaik, MM, Abraham, RS. A systematic review on predisposition to lymphoid (B and T cell) neoplasias in patients with Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity). Front Immunol 2019; 10: 777.CrossRefGoogle Scholar
Magdo, HS, Stillwell, TL, Greenhawt, MJ, et al. Immune abnormalities in Fontan protein-losing enteropathy: a case-control study. J Pediatr 2015; 167: 331–337.CrossRefGoogle ScholarPubMed
Strober, W, Wochner, RD, Carbone, PP, Waldmann, TA. Intestinal lymphangiectasia: a protein-losing enteropathy with hypogammaglobulinemia, lymphocytopenia and impaired homograft rejection. J Clin Invest 1967; 46: 1643–1656.CrossRefGoogle ScholarPubMed