Hindawi Case Reports in Immunology Volume 2019, Article ID 5476383, 5 pages https://doi.org/10.1155/2019/5476383 Case Report Castleman Disease in a Patient with Common Variable Immunodeficiency Luisa Ricciardi ,1 Fabiana Furci,1 Antonio Ieni ,2 and Antonio Macrì3 1 Allergy 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 Sof Tissue Sarcoma Program, Messina University Medical School Hospital, Messina, Italy Correspondence should be addressed to Luisa Ricciardi; [email protected] Received 3 January 2019; Revised 21 January 2019; Accepted 28 January 2019; Published 14 February 2019 Academic Editor: Necil K¨ut¨ukc¸¨uler Copyright © 2019 Luisa Ricciardi et al. Tis 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 immunodefciency (CVID) is a primary immunodefciency due to a disorder of the adaptive immune system which causes hypogammaglobulinemia and therefore an increased susceptibility to infection; noninfectious, infammatory conditions including systemic autoimmunity and lymphoproliferative complications are also commonly associated with CVID. Castleman disease (CD) is a systemic disease clinically characterized by difuse lymphadenopathy, splenomegaly, anemia, and systemic infammatory symptoms. Tis makes CD a great mimicker of more common benign and malignant masses in the neck, chest, abdomen, and pelvis. A novel case of primary immunodefciency (CVID) in a middle-aged woman, who developed multicentric CD (MDC) with splenomegaly, is described. Te 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 frst time in association with an adult case of CVID. Te 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. 1. Introduction is cure of the infections and chronic administration of immunoglobulins [1–3]. Common variable immunodefciency (CVID) is the most Castleman disease (CD), also known as angio-follicular common primary immunodefciency of young adolescents lymph node hyperplasia, is a rare disorder that can be unicen- and adults, which also afects children. It is characterized tric or multicentric. Unicentric Castleman disease (UCD) is by low antibody levels of immunoglobulins (IgG, IgA, IgM) localized and usually has an excellent prognosis. Multicentric that cause recurrent infections, especially bacterial, which Castleman disease (MCD) is a systemic disease clinically predominantly afect the respiratory and gastrointestinal characterized by difuse lymphadenopathy, splenomegaly, tract. In the lung, it is common to fnd granulomatous anemia, and systemic infammatory symptoms [4, 5]. Tis lymphocytic interstitial lung disease (GLILD) for which it makes MCD a great mimicker of more common benign and is necessary to make diferential diagnosis with lymphoma. malignant masses in the neck, chest, abdomen, and pelvis as Tese granulomatous lymphoid aggregates may also be found MCD masses commonly raise the suspicion of lymphoma, at other sites. In patients with CVID, there is a greater risk paraganglioma, metastatic adenopathy, solid parenchymal of autoimmune diseases, lymphomas, and other neoplasms or neuroendocrine tumors, and infectious or infammatory of the gastrointestinal tract. Te treatment of this pathology diseases [6]. 2 Case Reports in Immunology MCD is associated with an increased risk of developing spillage in the right and lef iliac fossa. A thoracic-abdominal malignancies [7]. CT with contrast medium showed the presence in both lungs It may manifest itself in two forms, and patients may of numerous occurrences of parenchymal thickening with present with either an indolent disease and very slow nodular appearance, some confuent, with irregular mor- progression, or an acute, fulminant disease; it has been phology, and contours. Te examination also highlighted the reported to occur in HIV patients who typically have a presence of bronchiectasis. Numerous paraaortic and iliac- simultaneous infection of human herpes virus 8 (HHV-8) obturatorlymphnodeswithashortaxisofabout12mmwere [8]. identifed. Other lymph nodes were identifed in the celiac site Nevertheless, the term MCD encompasses several dis- and along the small gastric curvature. Te liver, increased in tinct lymphoproliferative disorders with diferent underlying volume, did not show focal lesions. Port vein ectasia (24 mm) disease pathogenesis; even histopathological features are and splenic vein ectasia (25 mm) were highlighted (Figure 1). diverse as they are seen in diferent clinical variants of Surgical counselling recommended splenectomy. As it MCD and in reactive (autoimmune/infectious) and malig- was not an emergency surgery, in order to prevent any nant (lymphoma) context [9]. infectious surgical complication, IgG levels were maintained over 700 mg/dl for 2 months before splenectomy (Figure 2). A diagnosis of MCD is made by excisional biopsy of Spleen biopsies were performed, which showed a pre- afected lymph node tissue. Ten, a computed tomography dominant lymphocytic infltration (Figure 3). (CT) of the chest, abdomen, and pelvis should be performed to investigate the presence, or not, of adenopathy and A further thoracic-abdominal CT scan was performed splenomegaly. Nodal lesions in MCD more closely resemble three months afer surgery, which showed a reduced size of reactive or neoplastic nodal disease and calcifcations are the numerous paraaortic and iliac-obturator lymph nodes uncommon; intralesional necrosis or fbrosis may cause a with a short axis of about 8 mm. heterogenous appearance [5, 10]. We present the frst clinical case of a patient with CVID 3. Discussion who also developed MCD. CVID is a pathology which includes diferent phenotype presentations. Among the various phenotypes, the clinical 2. Case Report case presented represents one of considerable importance. A 51-year-old woman was diagnosed with CVID since Tis phenotype of CVID and Castleman’s disease is charac- 2000. Diagnosis was reached afer her having contracted terized by recurrent or chronic infections, lymphoid nodular two episodes of pneumonia and developing chronic diar- hyperplasia, hepatosplenomegaly, progressive chronic lung rhea. IVIG treatment was delivered every 45 days (4 gr/kg). disease with bronchiectasis, and increased risk of lymphoma Patient’s IgG levels reached normal blood levels (> 700 mg/dl) [2, 11]. Furthermore, the case we describe is the frst case with good clinical conditions. Since 2012, due to patient’s of adult CVID associated with Castleman’s disease as in personal reasons, IgG levels were not correctly kept within literature only a pediatric case was previously reported [12]. normal ranges; in 2017, the patient developed bilateral lat- Te case evolution is suggestive for a role of hypogam- erocervical lymph nodes (1 subtributary lymph node of maglobulinemia in the development of MCD as a CT 6.5 mm), lymph nodes in the mediastinal space (3.5 mm), and scan afer six months of correct IVIG treatment showed a splenomegaly. Histological examination on supraclavicular decrease in size of lymph nodes. Terefore, we hypothesize and abdominal lymph node biopsies was negative for neo- that the multicentric adenopathy with splenomegaly was plasm. Clinical signs of fatigue, fevers, and night sweats as a consequence of inappropriate treatment of CVID. Te well as anemia elevated CRP levels, and hepatosplenomegaly constant low levels of blood immunoglobulins most likely, was present. Te patient was diagnosed with MCD and as already reported, hyperstimulated the immune system referred to our clinical immunology unit due to severe causing a lymphoproliferative disorder with adenopathy and hypogammaglobulinemia and splenomegaly. splenomegaly [13]. Blood count detected hypochromic microcytic anemia, Idiopathic MCD, usually diagnosed afer excision biopsy mild neutropenia, and thrombocytopenia. Te study of lym- and comprehensive work-up of symptomatic lymph node phocyte subpopulations showed an inverted CD4/CD8 T- masses, has been reported to be characterized by an exagger- + cell ratio due to the numerically expansion of CD8 T-cells. ated systemic infammatory response secondary to a cytokine Immunoglobulin levels were low: IgG 345, IgA 2, and IgM storm involving Interleukin-6 (IL-6). 4 mg/dl. Wright agglutination test, markers of hepatitis B, A therapeutic approach for MCD is, in fact, anti-IL-6 hepatitis C, HIV, HHV8, tumor markers, serum and urine therapy siltuximab [14]. immunofxation, and fecal antigen H. Pylori were normal. Surgeons may also have an important role in the diagnos- IVIG treatment was started at 5 g/Kg maintaining IgG ticwork-upofMCD[15];inthecasewedescribe,MCDwith levels > 700 mg/dl as well as i.v. iron