Castleman Disease in a Patient with CommonVariable Immunodeficiency

Common variable immunodeficiency (CVID) is a primary immunodeficiency due to a disorder of the adaptive immunesystem which causes hypogammaglobulinemia and therefore an increased susceptibility to infection; noninfectious, inflammatoryconditions including systemic autoimmunity and lymphoproliferative complications are also commonly associated with CVID.Castleman disease (CD) is a systemic disease clinically characterized by diffuse lymphadenopathy, splenomegaly, anemia, andsystemic inflammatory symptoms. This makes CD a great mimicker of more common benign and malignant masses in theneck, chest, abdomen, and pelvis. A novel case of primary immunodeficiency (CVID) in a middle-aged woman, who developedmulticentric CD (MDC) with splenomegaly, is described. The authors suggest that the onset of MCD and of the correlatedsplenomegaly was due to incorrect management of the hypogammaglobulinemia as immunoglobulin G (IgG) levels were not keptwithin normal ranges. Correct management of the hypogammaglobulinemia allowed splenectomy to be performed without anyinfectious surgical complications. MCD is reported for the first time in association with an adult case of CVID. The above reportedcase highlights the need for a timely correct diagnosis and treatment of CVID to avoid complications, which could cause recourseto splenectomy, such as in our case or development of malignancies