Low Platelet Level May be a Predictor for Mortality in Adult Patients with Common Variable Immune Deficiency

Yıl 2021, Cilt: 2 Sayı: 3, 153 - 160, 22.09.2021

Gökhan Aytekin , Fatih Çölkesen , Eray Yıldız Şevket Arslan , Ahmet Çalışkaner

Öz

Background: Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency in adults. We, therefore, aimed to reveal mortality rates, causes of mortality in CVID patients, as well as demographic and clinical characteristics and differences of survived and dead CVID patients

Methods: The study group included 50 patients [(Female: 23 (46%), Male: 27 (54%)] with CVID, who were followed up on a regular basis for a period of ten years (115.18 ± 80.74 months).

Results: Diagnostic delay was 84 (0-360) months, and the mean follow-up time was 115.18 ± 80.74 months. The most common clinical presenting complaints were frequent and recurrent infections and pneumonia. At diagnosis, serum IgG levels were 1.72 (0.33 – 6.90) g/L. The overall survival rate of the patients during the follow-up time was 88%. As a result of univariate Cox regression analysis, platelet count was determined to be an independent risk factor for mortality in CVID patients (Hazard ratio, HR: 0.990, 95% confidence interval, CI: 981-0.999, p: 0.025). When the patients were classified according to mean platelet counts (platelets < 207770/mm3 and platelets > 207770/mm3), the mortality rate in the patient group with platelets < 207770/mm3 was determined to be statistically significantly higher compared to the patient group with platelets > 207770/mm3 (log-rank: 0.013).

Conclusions: Clinicians dealing with this patient group should remember that immune dysregulation and low platelet count are independent risk factors for mortality and they should remarkably follow up patients with low platelet count closely.

Anahtar Kelimeler

Platelets, Common Variable Immune Deficiency, Mortality.

Kaynakça

• 1. Sullivan KE, Puck JM, Notarangelo LD, Fuleihan R, Caulder T, Wang C, et al. USIDNET: a strategy to build a community of clinical immunologists. J Clin Immunol. 2014;34(4):428-35.
• 2. Bonilla FA, Barlan I, Chapel H, Costa-Carvalho BT, Cunningham- Rundles C, de la Morena MT, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract. 2016;4(1):38-59.
• 3. Picard C, Bobby Gaspar H, Al-Herz W, Bousfiha A, Casanova JL, Chatila T, et al. International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity. J Clin Immunol. 2018;38(1):96-128.
• 4. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D, Grimbacher B, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008;112(2):277-86.
• 5. Aghamohammadi A, Pouladi N, Parvaneh N, Yeganeh M, Movahedi M, Gharagolou M, et al. mortality and morbidity in common variable immunodeficiency. J Trop Pediatr. 2007;53(1):32-8.
• 6. Cunningham-Rundles C. How I treat common variable immune deficiency. Blood. 2010;116(1):7-15.
• 7. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999;92(1):34-48.
• 8. Kokron CM, Errante PR, Barros MT, Baracho GV, Camargo MM, Kalil J, et al. Clinical and laboratory aspects of common variable immunodeficiency. An Acad Bras Cienc. 2004;76(4):707-26.
• 9. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C.Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650-7.
• 10. Gathmann B, Mahlaoui N, Ceredih, Gerard L, Oksenhendler E, Warnatz K, et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134(1):116-26.
• 11. Feuille EJ, Anooshiravani N, Sullivan KE, Fuleihan RL, Cunningham- Rundles C. Autoimmune Cytopenias and Associated Conditions in CVID: a Report From the USIDNET Registry. J Clin Immunol. 2018;38(1):28-34.
• 12. Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27(3):308- 16.
• 13. Quinti I, Agostini C, Tabolli S, Brunetti G, Cinetto F, Pecoraro A, et al. Malignancies are the major cause of death in patients with adult onset common variable immunodeficiency. Blood. 2012;120(9):1953-4.
• 14. Boileau J, Mouillot G, Gerard L, Carmagnat M, Rabian C, Oksenhendler E, et al. Autoimmunity in common variable immunodeficiency: correlation with lymphocyte phenotype in the French DEFI study. J Autoimmun. 2011;36(1):25-32.
• 15. Patuzzo G, Barbieri A, Tinazzi E, Veneri D, Argentino G, Moretta F, et al. Autoimmunity and infection in common variable immunodeficiency (CVID). Autoimmun Rev. 2016;15(9):877 82.
• 16. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2019;123(5):454-60.
• 17. Aytekin G, Çölkesen F, Yıldız E, Arslan S. Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency. Asthma Allergy Immunol. 2019;17:160-5.
• 18. Fischer A, Provot J, Jais JP, Alcais A, Mahlaoui N, members of the CFPIDsg. Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies. J Allergy Clin Immunol. 2017;140(5):1388-93 e8