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

Introduction: Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency in adults. We 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, and the effect of platelet counts on mortality in these patients. Materials and methods: The study group included 50 patients [(Female: 23 (46%), Male: 27 (54%)] with CVID, who were followed-up at 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. Of the patients; 30% had lymphopenia, 56% splenomegaly, and 60% bronchiectasis. At diagnosis, serum IgG levels were 1.72 (0.33 – 6.90) g/L and switched memory B cell percentages 1.70 (0 – 52.0). 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). As a result of multivariate Cox regression analysis; current age, BMI and neutrophil/lymphocyte ratio were not found to be independent predictors for mortality. When the patients were classified according to mean platelet counts, the mortality rate in the patient group with platelets 207770/mm3 (log-rank: 0.013). The most common causes of mortality were pneumonia and pneumonia-induced sepsis. Conclusion: Clinicians dealing with this patient group should remember that immune dysregulation and low platelet count are independent risk factors for mortality and they should particularly follow-up patients with low platelet count closely.