Overlap of lymphatic dysplasia in Fontan-associated protein-losing enteropathy and Mucosa-Associated Lymphoid Tissue (MALT lymphoma): implications for management of protein-losing enteropathy. 2020

Joshua K Meisner, and Sarah Koblick, and Emily B Walling, and Raja Rabah, and Kurt R Schumacher
Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA48109.

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.

UI MeSH Term Description Entries
D008221 Lymphoid Tissue Specialized tissues that are components of the lymphatic system. They provide fixed locations within the body where a variety of LYMPHOCYTES can form, mature and multiply. The lymphoid tissues are connected by a network of LYMPHATIC VESSELS. Lymphatic Tissue,Lymphatic Tissues,Lymphoid Tissues,Tissue, Lymphatic,Tissue, Lymphoid,Tissues, Lymphatic,Tissues, Lymphoid
D009092 Mucous Membrane An EPITHELIUM with MUCUS-secreting cells, such as GOBLET CELLS. It forms the lining of many body cavities, such as the DIGESTIVE TRACT, the RESPIRATORY TRACT, and the reproductive tract. Mucosa, rich in blood and lymph vessels, comprises an inner epithelium, a middle layer (lamina propria) of loose CONNECTIVE TISSUE, and an outer layer (muscularis mucosae) of SMOOTH MUSCLE CELLS that separates the mucosa from submucosa. Lamina Propria,Mucosa,Mucosal Tissue,Muscularis Mucosae,Mucous Membranes,Membrane, Mucous,Membranes, Mucous,Mucosae, Muscularis,Mucosal Tissues,Propria, Lamina,Tissue, Mucosal,Tissues, Mucosal
D011504 Protein-Losing Enteropathies Pathological conditions in the INTESTINES that are characterized by the gastrointestinal loss of serum proteins, including SERUM ALBUMIN; IMMUNOGLOBULINS; and at times LYMPHOCYTES. Severe condition can result in HYPOGAMMAGLOBULINEMIA or LYMPHOPENIA. Protein-losing enteropathies are associated with a number of diseases including INTESTINAL LYMPHANGIECTASIS; WHIPPLE'S DISEASE; and NEOPLASMS of the SMALL INTESTINE. Enteropathy, Exudative,Idiopathic Hypercatabolic Hypoproteinemia,Enteropathy, Protein-Losing,Protein-Losing Enteropathy,Enteropathies, Exudative,Enteropathies, Protein-Losing,Exudative Enteropathies,Exudative Enteropathy,Hypercatabolic Hypoproteinemia, Idiopathic,Hypercatabolic Hypoproteinemias, Idiopathic,Hypoproteinemia, Idiopathic Hypercatabolic,Hypoproteinemias, Idiopathic Hypercatabolic,Idiopathic Hypercatabolic Hypoproteinemias,Protein Losing Enteropathies,Protein Losing Enteropathy
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D042601 Lymphatic Vessels Tubular vessels that are involved in the transport of LYMPH and LYMPHOCYTES. Capillaries, Lymphatic,Lymphatic Capillaries,Lymphatic Venules,Venules, Lymphatic,Capillary, Lymphatic,Lymphatic Capillary,Lymphatic Venule,Lymphatic Vessel,Venule, Lymphatic,Vessel, Lymphatic,Vessels, Lymphatic
D018442 Lymphoma, B-Cell, Marginal Zone Extranodal lymphoma of lymphoid tissue associated with mucosa that is in contact with exogenous antigens. Many of the sites of these lymphomas, such as the stomach, salivary gland, and thyroid, are normally devoid of lymphoid tissue. They acquire mucosa-associated lymphoid tissue (MALT) type as a result of an immunologically mediated disorder. Lymphoma, Mucosa-Associated Lymphoid Tissue,MALT Lymphoma,Marginal Zone B-Cell Lymphoma,Lymphoma of Mucosa-Associated Lymphoid Tissue,Mucosa-Associated Lymphoid Tissue Lymphoma,Lymphoma of Mucosa Associated Lymphoid Tissue,Lymphoma, MALT,Lymphoma, Mucosa Associated Lymphoid Tissue,Lymphomas, MALT,MALT Lymphomas,Marginal Zone B Cell Lymphoma,Mucosa Associated Lymphoid Tissue Lymphoma
D018729 Fontan Procedure A procedure in which total right atrial or total caval blood flow is channeled directly into the pulmonary artery or into a small right ventricle that serves only as a conduit. The principal congenital malformations for which this operation is useful are TRICUSPID ATRESIA and single ventricle with pulmonary stenosis. Fontan Circuit,Fontan Circulation,Fontan Operation,Fontan Palliation,Hemi-Fontan Procedure,Bidirectional Cavopulmonary Shunt,Bidirectional Glenn Procedure,Bidirectional Glenn Shunt,Norwood Procedure, Stage 2,Norwood Procedure, Stage 3,Norwood Procedure, Stage II,Norwood Procedure, Stage III,Stage 2 Norwood Procedure,Stage 3 Norwood Procedure,Stage II Norwood Procedure,Stage III Norwood Procedure,Bidirectional Cavopulmonary Shunts,Bidirectional Glenn Procedures,Bidirectional Glenn Shunts,Cavopulmonary Shunt, Bidirectional,Circuit, Fontan,Circulation, Fontan,Glenn Procedure, Bidirectional,Glenn Shunt, Bidirectional,Hemi Fontan Procedure,Operation, Fontan,Palliation, Fontan,Procedure, Bidirectional Glenn,Procedure, Fontan,Procedure, Hemi-Fontan,Shunt, Bidirectional Cavopulmonary

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