Asthma Allergy Immunology

Asthma Allergy Immunology

2019, Vol 17, Num, 1     (Pages: 057-060)

Successful Immunoglobulin Replacement with Subcutaneous Immunoglobulin Therapy in a Patient with Primary Intestinal Lymphangiectasia

Gökhan AYTEKİN 1, Fatih ÇÖLKESEN 1, Ömür ARDENİZ 2, Zafer CALIŞKANER 1,

1 Department of Allergy and Clinical Immunology, Necmettin Erbakan University, Meram School of Medicine, Konya, Turkey
2 Department of Allergy and Clinical Immunology, Ege University, Health Practice and Research Center (Ege University Hospital), Izmir, Turkey

DOI: 10.21911/aai.442
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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.

Keywords : Primary Intestinal Lymphangiectasia, secondary hypoglobulinemia, subcutaneous immunoglobulin