CASE REPORT
Common variable immunodeficiency in an 11-year-old girl – a case report
 
More details
Hide details
1
Department of Paediatrics and Allergy, Medical University, Lodz, Copernicus Memorial Hospital, Korczak Paediatric Centre, Lodz, Poland
 
 
Corresponding author
Joanna Pawłowska   

Department of Pediatrics and Allergy, Medical University of Lodz, Copernicus Memorial Hospital, Korczak Pediatric Centre, Lodz., Piłsudskiego 71, 90-329, Lodz, Poland
 
 
J Pre Clin Clin Res. 2020;14(1):14-17
 
KEYWORDS
TOPICS
ABSTRACT
Common variable immunodeficiency (CVID) is one of the most prevalent, heterogenous primary immunodeficiency syndromes in children and adults. Besides impaired immunoglobulin production by B cells, abnormalities in almost all components of the immune system have been desribed in CVID. The clinical pictures of CVID includes acute and chronic infections, inflammatory and autoimmune diseases, and can be associated with an increased incidence of cancer and lymphoma. Due to the plurality of clinical manifestations and different ages of occurance, diagnosis of CVID is difficult and belated in relation to primary symptoms. Patients with CVID require an integrated interprofessional team approach to minimalize complications of the disease and to improve patient outcomes. The article presents the clinical case of 11-yearold girl diagnosed with CVID in day-to-day medical practice.
REFERENCES (27)
1.
Grześk E, et al. Common variable immunodeficiency disorders – diseases with many faces. Pediatr Pol 2018; 93(2): 186–191. doi: https:// doi.org/10.5114/polp.2….
 
2.
Cooper CJ, Said S, Quansah R, et al. Pneumococcal meningitis in a young adult female with common variable immunodeficiency. Am J Case Rep 2013; 14: 471–475.
 
3.
Bonilla FA et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract 2016; 4: 38–59. doi: 10.1016/j.jaip.2015.07.025.
 
4.
Tseng CW, Lai KL, Chen DY, Lin CH, Chen HH. The incidence and prevalence of common variable immunodeficiency disease in Taiwan, a population-based study. PLoS ONE 2015; 10: e0140473.
 
5.
Jolles S. The variable in common variable immunodeficiency: a disease of complex phenotypes. J Allergy Clin Immunol Pract. 2013; 1: 545–556.
 
6.
Abolhassani H, Sagvand BT, Shokuhfar T, et al. A review on guidelines for management and treatment of common variable immunodeficiency. Expert Rev Clin Immunol. 2013; 9: 561–574.
 
7.
Szczawinska-Poplonyk A, Tapolska-Jozwiak K, Samara H. The B-cell compartment in antibody-deficient infants and young children – developing common variable immunodeficiency or transient immune maturation? Ital J Pediatr. 2016; 42: 71.
 
8.
Lankisch P, Schiffner J, Ghosh S, et al. The Duesseldorf warning signs for primary immunodeficiency: is it time to change the rules? J Clin Immunol. 2015; 35: 273–279.
 
9.
Modell V, Quinn J, Orange J, Notarangelo LD, Modell F. Primary immunodeficiencies worldwide: an updated overview from the Jeffrey Modell Centers Global Network. Immunol Res. 2016; 64: 736–53.
 
10.
Cunningham-Rundles Ch. Common Variable Immune Deficiency: Dissection of the Variable. Immunol Rev. 2019 January; 287(1): 145–161. doi: 10.1111/imr.12728.
 
11.
Gereige JD, Maglione PJ. Current Understanding and Recent Developments in Common Variable Immunodeficiency Associated Autoimmunity. Front Immunol. 2019; 10: 2753. doi: 10.3389/ fimmu.2019.02753.
 
12.
Edgar D, Ehl S. ESID Registry – Working Definitions for Clinical Diagnosis of PID. 2016. p. 1–33.
 
13.
 
14.
Cunningham-Rundles Ch. The many faces of common variable immunodeficiency. Hematology Am Soc Hematol Educ Program. 2012; 2012: 301–305. doi: 10.1182/asheducation-2012.1.301.
 
15.
Conaway E. Novel variant of common variable immunodeficiency. BMJ Case Rep 2017 May 27. doi: 10.1136/bcr-2017-219655.
 
16.
Cunningham-Rundles C. The many faces of common variable immunodeficiency. Hematology Am Soc Hematol Educ Program 2012; 2012: 301–305.
 
17.
Genre J, Errante PR, Kokron CM, Toledo-Barros M, Câmara NOS, Rizzo LV. Reduced frequency of CD4+CD25HIGHFOXP3+ cells and diminished FOXP3 expression in patients with common variable immunodeficiency: a link to autoimmunity? Clin Immunol. 2009; 132: 215–21. doi: 10.1016/j.clim.2009.03.519.
 
18.
Patuzzo G, Barbieri A, Tinazzi E, et al. Autoimmunity and infection in common variable immunodeficiency (CVID). Autoimmun Rev. 2016; 15: 877–882. doi: 10.1016/j.autrev.2016.07.011.
 
19.
Arandi N, Mirshafiey A, Abolhassani H, Jeddi-Tehrani M, Edalat R, Sadeghi B, et al. Frequency and expression of inhibitory markers of CD4 + CD25 + FOXP3 + regulatory T cells in patients with common variable immunodeficiency. Scand J Immunol. 2013; 77: 405–12. doi: 10.1111/sji.12040.
 
20.
Azizi G, Tavakol M, Rafiemanesh H, et al. Autoimmunity in a cohort of 471 patients with primary antibody deficiencies. Expert Rev Clin Immunol. 2017; 13: 1099–1106.
 
21.
Agondi, et al. Allergic asthma in patients with common variable immunodeficiency. Allergy 2010; 65: 510–515.
 
22.
Dhalla F, da Silva SP, Lucas M. Review of gastric cancer risk factors in patients with common variable immunodeficiency disorders, resulting in a proposal for a surveillance programme. Clin Exp Immunol. 2011; 165: 1–7.
 
23.
Gathmann B, Mahlaoui N, Gérard L, et al. European Society for Immunodeficiencies Registry Working Party: Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014; 134: 116–126.
 
24.
Gobert D, Bussel JB, Cunningham-Rundles C, et al. Efficacy and safety of rituximab in common variable immunodeficiency associated immune cytopenias: a retrospective multicentre study on 33 patients. Br J Haematol. 2011; 155: 498–508.
 
25.
Pac M, Bernatowska E. Comprehensive activities to increase recognition of primary immunodeficiency and access to immunoglobulin replacement therapy in Poland. Eur J Pediatr. 2016; 175: 1099–1105.
 
26.
Mohammadinejad P, Aghamohammadi A, Abolhassani H, et al. Pediatric patients with common variable immunodeficiency: longterm follow-up. J Investig Allergol Clin Immunol. 2012; 22: 208–214.
 
27.
Kalicki B, et al. Common variable immunodeficiency – case report. Centr Eur J Immunol. 2013; 38(1): 141–148. DOI: 10.5114/ceji.2013.34373.
 
eISSN:1898-7516
ISSN:1898-2395
Journals System - logo
Scroll to top