Phenotypic profile among patients with Autoimmune Cytopenia’s in Common Variable Immunodeficiency

Introduction: Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency .(1) Autoimmune cytopenia’s (AIC) (autoimmune hemolytic anemia and/or thrombocytopenia) are the most common autoimmune complications seen in patients with CVID.(2)occurring among 4-20% of patients (3) Objectives: To determine Freiburg phenotype among CVID with AIC and its association with comorbidities. Methodology: We enroll 35 patients with the diagnostic of CVID according to the European Society of Immunodeficiency (ESID).Results the mean age of the population 29 years (4-86), regarding the distribution of sex 13 (35.2%) patients were men and 22(64.7%) were women. About AIC 21(60%) of the patients had any at the time of recruitment , 12(40%) female and 9 (30%) male; the most common was primary immune thrombocytopenia 20(57.1%) followed by hemolytic anemia 4(11.4%) . About the Freiburg classification, we found a predominant distribution of phenotype 1b 13(37.1%) in second place 1a 12(34.2%) of the total sample, however, the distribution by Freiburg phenotypes in patients with AIC was Freiburg 1a 10(33.3%) and Freiburg 1b 6(20%). Of the total sample, 11 (25%) patients had some AIC as their debut, the mean IgG concentration in those with autoimmune cytopenia’s was 355 (6-1433) mg/dl, IgA 22.71(1-187) mg/dl IgM 32.35(0-160) mg/dl vs IgG 174.5 (34-426) mg/dl IgA 67.6 (0-402)mg/dl and IgM 52.8(1-423) mg/dl among patients with no AIC. In patients with AIC at least one autoimmune comorbidity was observed in 21 (70%) finally the most common infectious complication associated with AIC was sinusitis 15(42.8%) followed by pneumonia and otitis 11(31.4%) of the cases Conclusion: In this descriptive study we find that among the patients with CVID in our center we found a predominance of females in AIC patients, they had higher IgG levels and the predominant Freiburg phenotype was 1a consistent with the literature and comorbidity.

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