title: Secondary hypogammaglobulinemia in Waldmann's disease treated with subcutaneous immunoglobulins.
abstract: Primary intestinal lymphangiectasia (PIL) is rare disorder characterized by congenital malformation or obstruction of intestinal lymphatic drainage; it is responsible for protein losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL management. The administration of intravenous immunoglobulins does not always lead to satisfactory plasma levels and therefore the replacement therapy with immunoglobulins is controversial. We describe here the case of a patient with PIL and severe hypogammaglobulinemia treated with immunoglobulins. The striking aspect of this case is the clinical and serological benefit obtained with the subcutaneous compared to the intravenous immunoglobulins administration.
authors.affiliation: Clinical Immunology and Allergy Unit Department of Medicine University of Verona 37134 Verona, Italy. E-mail: jeps.pz@gmail.com Phone: +39 045 812 4401 Fax: +39 045 802 7473.
authors.forename: G
authors.initials: G
authors.lastname: Patuzzo
authors.affiliation: Department of Medicine, University of Verona, Verona, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Tinazzi
authors.affiliation: Department of Medicine, University of Verona, Verona, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Micheletti
authors.affiliation: Immunology Area, Pediatric Hospital Bambino Gesù, Rome, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Puccetti
authors.affiliation: Department of Medicine, University of Verona, Verona, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Lunardi
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D018752:Diet, Fat-Restricted; D006801:Humans; D007074:Immunoglobulin G / Q000008:administration & dosage* / Q000097:blood; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000097:blood; D055104:Infusions, Subcutaneous; D008201:Lymphangiectasis, Intestinal / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D008209:Lymphedema / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D008297:Male; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014280:Triglycerides / Q000008:administration & dosage
chemical_list: C558471:Hizentra; D007074:Immunoglobulin G; D007155:Immunologic Factors; D014280:Triglycerides
keywords: Waldmann‘s disease; primary intestinal lymphangectasia; protein-losing enteropathy; secondary immunodeficiencies; subcutaneous immunoglobulins