Avtor/Urednik     Petric, Vlasta; Rotdajč, Romana; Šeruga, Maja
Naslov     Avtoimuni citopeniji pri bolniku z navadno spremenljivo imunsko pomanjkljivostjo
Prevedeni naslov     Autoimmune cytopenias related to common variable immunodeficiency
Tip     članek
Vir     Zdrav Vestn
Vol. in št.     Letnik 80, št. 9
Leto izdaje     2011
Obseg     str. 698-702
Jezik     slo
Abstrakt     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.
Deskriptorji     COMMON VARIABLE IMMUNODEFICIENCY
THROMBOCYTOPENIA
ANEMIA, HEMOLYTIC
IGG
IGA
ADULT
IGM
METHYLPREDNISOLONE