The Evaluation of Neutropenia in Common Variable Immune Deficiency Patients Publisher Pubmed



Ghorbani M¹ ; Fekrvand S¹ ; Shahkarami S^{1, 2} ; Yazdani R¹ ; Sohani M¹ ; Shaghaghi M^{1, 3} ; Hassanpour G⁴ ; Mohammadi J⁵ ; Negahdari B⁶ ; Abolhassani H^{1, 7} ; Aghamohammadi A¹
Source: Expert Review of Clinical Immunology Published:2019

Abstract

Objectives: Common variable immunodeficiency is a primary immunodeficiency disease characterized by hypogammaglobulinemia and heterogeneous clinical features. Neutropenia is a rare complication among CVID patients leading to a higher rate of infections and morbidity. Multiple factors (e.g. autoimmunity, infections, drugs and etc.) are found to underlie this complication. Methods: In the present study, demographic, clinical and laboratory data were compared between two groups of CVID patients with and without neutropenia. Results: Frequency of neutropenia was 8.1%. Infectious complications were the most prevalent clinical manifestations regardless of presence of neutropenia. However, candida infection and septicemia were significantly higher in neutropenic patients (p = 0.001 and p = 0.01, respectively). The most prominent clinical phenotypes of CVID patients with neutropenia were polyclonal lymphocytic infiltration and autoimmunity, both being considerably higher compared to the non-neutropenic group (p = 0.04 and p = 0.009, respectively). The mortality rate in neutropenic patients was higher than in patients without neutropenia (61.1 vs. 25.2%, p = 0.004). Conclusion: Although neutropenia is a rare complication among CVID patients, it is associated with frequent and severe clinical complications, including autoimmunity and lymphoproliferative conditions. Also, its accompaniment with higher mortality frequency in CVID patients indicates a need for more precise attention and consideration regarding specific treatment in neutropenic patients. © 2019, © 2019 Informa UK Limited, trading as Taylor & Francis Group.