Author/Editor		Petric, Vlasta; Rotdajč, Romana; Šeruga, Maja
Title		Avtoimuni citopeniji pri bolniku z navadno spremenljivo imunsko pomanjkljivostjo
Translated title		Autoimmune cytopenias related to common variable immunodeficiency
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 80, št. 9
Publication year		2011
Volume		str. 698-702
Language		slo
Abstract		Background: Common variable immunodeficiency disorders are characterised by defective antibody production leading to recurrent infections. Noninfective complications are a consequence of autoimmunity, granuloma and polyclonal lymphoid infiltration. We often detect autoimmune cytopenias before primary immunodefciency is confirmed. Patients and methods: We report a case of 39-year old man with recurrent respiratory infections, autoimmune thrombocytopenia and haemolytic anemia who had common varible immunodeficiency confirmed. He had a lack of serum IgG, IgA and IgM, bronchiectasis, lymphadenopathy, splenomegaly, hepatic granuloma, autoimmune gastritis with B12 deficiency and Evans syndrome. We treated autoimmune cytopenias with methylprednisolon and cyclosporine. After substitution therapy with intravenous immunoglobulin the frequency of espiratory infections decreased. Occurrence of diarrhea is suspected for enteropathy, however, hystologic identification is required. Because of patologically changed gastric mucosa and signs of polyclonal lymphoid infiltration, the patient is at high risk for malignancy and the outcome of the disease remains unpredictable. Conclusions: Generally, we discover common variable immunodeficiency at management of noninfective complications, in wich intravenous immunoglobulin are not effective. Autoimmune cytopenias and some other complications are successfully treated with glucocorticoids. Careful monitorig of these patients is important because of a high risk for malignancy.
Descriptors		COMMON VARIABLE IMMUNODEFICIENCY THROMBOCYTOPENIA ANEMIA, HEMOLYTIC IGG IGA ADULT IGM METHYLPREDNISOLONE