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Case Reports in Immunology
Volume 2019, Article ID 5476383, 5 pages
Case Report

Castleman Disease in a Patient with Common Variable Immunodeficiency

1Allergy and Clinical Immunology Unit, Department of Clinical and Experimental Medicine, Medical School Hospital G. Martino, University of Messina, Messina, Italy
2Department of Human Pathology in Adult and Developmental Age “Gaetano Barresi”, Unit of Pathological Anatomy, University Medical School Hospital G. Martino, University of Messina, Messina, Italy
3Peritoneal Surface Malignancy and Soft Tissue Sarcoma Program, Messina University Medical School Hospital, Messina, Italy

Correspondence should be addressed to Luisa Ricciardi; ti.eminu@idraiccirl

Received 3 January 2019; Revised 21 January 2019; Accepted 28 January 2019; Published 14 February 2019

Academic Editor: Necil Kütükçüler

Copyright © 2019 Luisa Ricciardi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Common variable immunodeficiency (CVID) is a primary immunodeficiency due to a disorder of the adaptive immune system which causes hypogammaglobulinemia and therefore an increased susceptibility to infection; noninfectious, inflammatory conditions 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, and systemic inflammatory symptoms. This makes CD a great mimicker of more common benign and malignant masses in the neck, chest, abdomen, and pelvis. A novel case of primary immunodeficiency (CVID) in a middle-aged woman, who developed multicentric CD (MDC) with splenomegaly, is described. The authors suggest that the onset of MCD and of the correlated splenomegaly was due to incorrect management of the hypogammaglobulinemia as immunoglobulin G (IgG) levels were not kept within normal ranges. Correct management of the hypogammaglobulinemia allowed splenectomy to be performed without any infectious surgical complications. MCD is reported for the first time in association with an adult case of CVID. The above reported case highlights the need for a timely correct diagnosis and treatment of CVID to avoid complications, which could cause recourse to splenectomy, such as in our case or development of malignancies.