Hypogammaglobulinaemia: cumulative experience in 49 patients in a tertiary care institution.

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Title:	Hypogammaglobulinaemia: cumulative experience in 49 patients in a tertiary care institution.
Author(s):	Hilst, J.C.H. van der ; Smits, B.W. ; Meer, J.W.M. van der
Publication year:	2002
Source:	Netherlands Journal of Medicine, vol. 60, iss. 3, (2002), pp. 140-147
ISSN:	0300-2977
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : http://hdl.handle.net/2066/147364
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Subject:	The role of cytokines in the pathophysiology of febrile illnesses and in host defense against infections Pathophysiology of Brain and Behaviour De rol van cytokinen in de pathofysiologie van koortsende ziekten en in de afweer tegen infecties Pathofysiologie van Hersenen en Gedrag
Organization:	Internal Medicine Neurology
Journal title:	Netherlands Journal of Medicine
Volume:	vol. 60
Issue:	iss. 3
Page start:	p. 140
Page end:	p. 147
Abstract:	In this paper, clinical data of 49 adult patients with agammaglobulinaemia (syn. hypogammaglobulinaemia), 15 cases of X-linked agammaglobulinaemia (XLA) and 34 of common variable immunodeficiency (CVID) are reviewed. Although immunoglobulin substitution largely abolished life-threatening respiratory tract infections, considerable infectious and non-infectious morbidity was still encountered in these patients. Almost all patients suffered from chronic or recurrent upper and lower airway infections, mainly caused by Haemophilus influenzae and pneumococci. The lower respiratory tract infections led to cumulative damage to the respiratory tract, especially in XLA patients. Also the incidence of infections outside the respiratory tract (giardiasis, Campylobacter jejuni infections) was more common in XLA patients than in CVID patients. Nodular lymphoid hyperplasia was only found in CVID. A variety of other non-infectious complications were seen especially in CVID. Neoplastic complications occurred in nine patients (two cases of thymoma, two colorectal cancer, one gastric carcinoma, two haematological malignancies, two cases of skin cancer). Six patients died (five XLA patients and one CVID patient, from infectious and non-infectious causes).