Characteristics of the patients followed with the diagnosis of common variable immunodeficiency and the complications

被引:9
|
作者
Erdem, Semiha Bahceci [1 ]
Gulez, Nesrin [1 ]
Genel, Ferah [1 ]
Karaman, Salt [1 ]
Nacaroglu, Hikmet T. [2 ]
机构
[1] Dr Behcet Uz Children Dis & Surg Training & Res H, Dept Pediat Allergy & Immunol, TR-35220 Izmir, Turkey
[2] Istambul Medipol Univ, Dept Pediat Allergy & Immunol, Istanbul, Turkey
关键词
primary immunodeficiency; hypogammaglobulinemia; common variable immunodeficiency; TACI; SELECTIVE IGA DEFICIENCY; IMMUNE-DEFICIENCY; LUNG-DISEASE; DISORDERS; CHILDREN; MANIFESTATIONS; MANAGEMENT; MUTATIONS; FAMILY;
D O I
10.5114/ceji.2019.87060
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Introduction: In this study, we aimed to retrospectively evaluate the clinical and laboratory findings and complications of 28 common variable immunodeficiency (CVID) patients. Material and methods: The clinical features and laboratory data of 28 CVID patients were evaluated. Results: Nineteen patients were male. In 53.5% of the cases, complications included inflammatory bowel disease, cytopenia, bronchiectasis, granulomatous lymphocytic interstitial lung disease (ILD) and asthma. In their immunological evaluations, IgG, IgM, and IgA mean values were 474.8 +/- 214.1 mg/dl; 56.7 +/- 41.9 mg/dl; 35.3 +/- 58.2 mg/dl, respectively, and the vaccine response was positive in 64.2% of the cases. In all age groups, absolute lymphocyte counts, naive (CD19+IgD+27-), nonswitch (CD19+IgD-27+) memory B cells were numerically higher when compared to the data of healthy children; however, although switch memory (CD19+IgD+27+) B cells were proportionally low in the 4-8 and 12-18 age groups, they were low both numerically and proportionally in the 8-12 age group. No statistically significant difference was found between the cases with complications and without complications. But the cases with pulmonary complications were compared within the group, the CD8 ratio was high but the IgA level was low in patients with bronchiectasis and CD3 was numerically and proportionally low in the cases with ILD compared to others. According to the Paris classification, 11/27 (40.7%) of the cases, 3/27 (11.1%) of them and 13/27 (48.2%) of them were evaluated as MB0, MB1, and MB2, respectively. Conclusions: In genetic studies, TACI (trans-membrane activator and calcium-modulating cyclophilin ligand interactor - TNFRSF13B) mutation was found positive in 25% of the cases.
引用
收藏
页码:119 / 126
页数:8
相关论文
共 50 条
  • [31] Common Variable Immunodeficiency Diagnosis, Management, and Treatment
    Abbott, Jordan K.
    Gelfand, Erwin W.
    IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA, 2015, 35 (04) : 637 - +
  • [32] Bronchial Asthma and Bronchial Hyperresponsiveness and Their Characteristics in Patients with Common Variable Immunodeficiency
    Milota, Tomas
    Bloomfield, Marketa
    Parackova, Zuzana
    Sediva, Anna
    Bartunkova, Jirina
    Horvath, Rudolf
    INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY, 2019, 178 (02) : 192 - 200
  • [33] Lymphocyte characteristics in children with common variable immunodeficiency
    van de Ven, Annick A. J. M.
    van de Corput, Lisette
    van Tilburg, Cornelis M.
    Tesselaar, Kiki
    van Gent, Rogier
    Sanders, Elisabeth A. M.
    Boes, Marianne
    Bloem, Andries C.
    van Montfrans, Joris M.
    CLINICAL IMMUNOLOGY, 2010, 135 (01) : 63 - 71
  • [34] Autoimmune disorders associated with common variable immunodeficiency: prediction, diagnosis, and treatment
    Yazdanpanah, Niloufar
    Rezaei, Nima
    EXPERT REVIEW OF CLINICAL IMMUNOLOGY, 2022, 18 (12) : 1265 - 1283
  • [35] Sarcoidosis and Common Variable Immunodeficiency: Similarities and Differences
    Verbsky, James W.
    Routes, John M.
    SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE, 2014, 35 (03) : 330 - 335
  • [36] Common variable immunodeficiency: a multifaceted and puzzling disorder
    Bergbreiter, Astrid
    Salzer, Ulrich
    EXPERT REVIEW OF CLINICAL IMMUNOLOGY, 2009, 5 (02) : 167 - 180
  • [37] Chronic Diarrhea in Common Variable Immunodeficiency: a Case Series and Review of the Literature
    Pecoraro, Antonio
    Nappi, Liliana
    Crescenzi, Ludovica
    D'Armiento, Francesco P.
    Genovese, Arturo
    Spadaro, Giuseppe
    JOURNAL OF CLINICAL IMMUNOLOGY, 2018, 38 (01) : 67 - 76
  • [38] Autoimmune and Lymphoproliferative Complications of Common Variable Immunodeficiency
    Maglione, Paul J.
    CURRENT ALLERGY AND ASTHMA REPORTS, 2016, 16 (03) : 1 - 10
  • [39] Autoimmunity in common variable immunodeficiency
    Agarwal, Shradha
    Cunningham-Rundles, Charlotte
    ANNALS OF ALLERGY ASTHMA & IMMUNOLOGY, 2019, 123 (05) : 454 - 460
  • [40] Adult Common Variable Immunodeficiency
    Dong, Jing
    Liang, Hongda
    Wen, Dawei
    Wang, Jibo
    AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 2016, 351 (03) : 239 - 243