Secondary hypogammaglobulinemia in waldmann’s disease treated with subcutaneous immunoglobulins

Giuseppe Patuzzo, E. Tinazzi, M. Micheletti, A. Puccetti, C. Lunardi

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)55-57
Number of pages3
JournalEuropean Annals of Allergy and Clinical Immunology
Volume48
Issue number2
Publication statusPublished - Mar 1 2016

Keywords

  • Primary intestinal lymphangectasia
  • Protein-losing enteropathy
  • Secondary immunodeficiencies
  • Subcutaneous immunoglobulins
  • Waldmann’s disease

ASJC Scopus subject areas

  • Immunology and Allergy

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