Primary intestinal lymphangiectasia is a rare disorder which characterized by impaired small intestinal lymph drainage. There is loss of proteins from dilated lymphatic channels located in the mucosa, submucosa or subserosa which is results with loss of gammaglobulins and lymphocytes, leading to impaired humoral and cellular immunity. Herein, we present a 61-year-old patient with immunodeficiency secondary to Primary intestinal lymphangiectasia (PIL), in whom we could attain effective and stable IgG levels only by subcutaneous IgG replacement rather than intravenous IgG. Our experience suggests that Subcutaneous Immunoglobulin (SCIG) replacement resulted in more stable levels of IgG in the presented patient with PIL. We concluded that SCIG should be the preferred route of immunoglobulin replacement therapy in secondary hypogammaglobulinemia due to protein losing enteropathy, especially in PIL.
1. Blau IW, Conlon N, Petermann R, Nikolov N, Plesner T. Facilitated subcutaneous immunoglobulin administration (fSCIg): A new treatment option for patients with secondary immune deficiencies. Expert Rev Clin Immunol 2016;12(7):705- 11.
2. Windegger TM, Lambooy CA, Hollis L, Morwood K, Weston H, Fung YL. Subcutaneous immunoglobulin therapy for hypogammaglobulinemia secondary to malignancy or related drug therapy. Transfus Med Rev 2017;31(1):45-50.
3. Streu E. Subcutaneous immunoglobulin in oncology clinical practice. Clin J Oncol Nurs 2016;20(4):437-9.
4. Spadaro G, Pecoraro A, De Renzo A, Della Pepa R, Genovese A. Intravenous versus subcutaneous immunoglobulin replacement in secondary hypogammaglobulinemia. Clin Immunol 2016;166-167:103-4.
5. Patuzzo G, Tinazzi E, Micheletti M, Puccetti A, Lunardi C. Secondary hypogammaglobulinemia in Waldmann’s disease treated with subcutaneous immunoglobulins. Eur Ann Allergy Clin Immunol 2016;48(2):55-7.
6. Lin JH, Roberts RL. Subcutaneous immunoglobulin infusions for treatment of a child with severe protein-losing enteropathy. J Allergy Clin Immunol 2004;113(Suppl 2):S42
7. Shah SN, Todoric K, Tarrant TK. Improved outcomes on subcutaneous IgG in patients with humoral immunodeficiency and co-morbid bowel disease. Clin Case Rep Rev 2015;1(7):151- 2.
8. Krishnarajah G, Lehmann JK, Ellman B, Bhak RH, DerSarkissian M, Leader D Jr, et al. Evaluating dose ratio of subcutaneous to intravenous immunoglobulin therapy among patients with primary immunodeficiency disease switching to 20% subcutaneous immunoglobulin therapy. Am J Manag Care 2016;22:s475-s81.
9. Berger M. Choices in IgG replacement therapy for primary immune deficiency diseases: subcutaneous IgG vs. intravenous IgG and selecting an optimal dose. Curr Opin Allergy Clin Immunol 2011;11(6):532-8.
10. 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(10):1643-56.
11. Lynn J, Knight AK, Kamoun M, Levinson AI. A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts. J Allergy Clin Immunol 2004;114(2):409-14.
12. Bouhnik Y, Etienney I, Nemeth J, Thevenot T, Lavergne-Slove A, Matuchansky C. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. Gut 2000;47(2):296-300.
13. Jabeen SA, Murthy A, Kandadai RM, Meena AK, Borgohain R, Uppin MS. Cryptoccocal menigitis as a primary manifestation in a patient with intestinal lymphangictasia. Ann Indian Acad Neurol 2012;15(3):218-20.
14. Heresbach D, Raoul JL, Genetet N, Noret P, Siproudhis L, Ramee MP, et al. Immunological study in primary intestinal lymphangiectasia. Digestion 1994;55(1):59-64.
15. Huber X, Degen L, Muenst S, Trendelenburg M. Primary intestinal lymphangiectasia in an elderly female patient: A case report on a rare cause of secondary immunodeficiency. Medicine (Baltimore) 2017;96(31):e7729.