Resolution of Hypersplenism and Pancytopenia in Common Variable Immunodeficiency Patient After Partial Splenic Artery Embolization: A Case Report and Review of Literature

Abstract

Common variable immunodeficiency (CVID) is the second most prevalent primary immunodeficiency disorder following selective immunoglobulin A (IgA) deficiency but it is more complicated clinically. It causes a wide spectrum of symptoms and signs affecting several systems of the body. CVID is mostly a humoral immunodeficiency with cell-mediated immunity being affected in some patients leading to both autoimmunity and malignancy adding to the immunodeficiency, which makes the management of these patients very complicated. Gastrointestinal tract (GIT) and bone marrow are commonly affected organs by both autoimmunity and malignancy in the context of CVID. In this paper, we present a case of CVID who presented with pancytopenia secondary to hypersplenism and failed both intravenous immunoglobulins (IVIG) and steroids treatment. She underwent partial splenic artery embolization as an alternative procedure to total splenectomy to preserve some splenic function and avoid surgical total splenectomy. Partial splenic artery embolization leads to dramatic improvement in platelets, hemoglobin and white cell count after the procedure without further compromising her immune system.



J Hematol. 2015;4(2):174-177
doi: http://dx.doi.org/10.14740/jh168w