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J Clin Immunol. 2015 Aug;35(6):589-94. doi: 10.1007/s10875-015-0189-6. Epub 2015 Aug 29.

Profound Reversible Hypogammaglobulinemia Caused by Celiac Disease in the Absence of Protein Losing Enteropathy.

Author information

1
Department of Clinical Immunology, Auckland Hospital, Park Rd, Grafton, Auckland, 1010, New Zealand. immunology@xtra.co.nz.
2
Department of Virology and Immunology, Auckland Hospital, Park Rd, Grafton, Auckland, 1010, New Zealand. immunology@xtra.co.nz.
3
Department of Virology and Immunology, Auckland Hospital, Park Rd, Grafton, Auckland, 1010, New Zealand.
4
Department of Clinical Immunology, Auckland Hospital, Park Rd, Grafton, Auckland, 1010, New Zealand.
5
Department of Histopathology, Diagnostic Medlab, Harrison Rd, Ellerslie, Auckland, 1051, New Zealand.

Abstract

When patients with hypogammaglobulinemia are encountered, a vigorous search should be undertaken for secondary treatable causes. Here we describe the first case of a patient with severe asymptomatic hypogammaglobulinemia where the underlying cause was undiagnosed celiac disease. A strict gluten free diet resulted in resolution of her mild long-standing abdominal symptoms and correction of her hypogammaglobulinemia. There was corresponding improvement in her duodenal histology and normalisation of her celiac serology. Protein losing enteropathy was unlikely to have been the mechanism of her profound hypogammaglobulinemia, as her albumin was within the normal range and she had a normal fecal alpha 1 antitrypsin level. Application of the Ameratunga et al. (2013) diagnostic criteria was helpful in confirming this patient did not have Common Variable Immunodeficiency Disorder (CVID). Celiac disease must now be considered in the differential diagnosis of severe hypogammaglobulinemia. There should be a low threshold for undertaking celiac serology in patients with hypogammaglobulinemia, even if they have minimal symptoms attributable to gut disease.

KEYWORDS:

CVID; Celiac disease; hypogammaglobulinemia

PMID:
26318181
DOI:
10.1007/s10875-015-0189-6
[Indexed for MEDLINE]

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