Pulmonary Manifestations of Plasma Cell Type Idiopathic Multicentric Castleman Disease: A Clinicopathological Study in Comparison with IgG4-Related Disease

Plasma cell type idiopathic multicentric Castleman disease (PC-iMCD) occasionally manifests as parenchymal lung disease. This study aimed to elucidate the detailed clinicopathological features of lung lesions in PC-iMCD and compare the findings with those in immunoglobulin (Ig) G4-related disease (IgG4-RD), the most difficult differential diagnosis of PC-iMCD. We analyzed the clinicopathological findings and immunohistochemical expression patterns of interleukin-6 (IL-6) and Igs in lung specimens from 16 patients with PC-iMCD and 7 patients with IgG4-RD. Histologically, pulmonary PC-iMCD could not be differentiated from IgG4-RD based on lesion distribution patterns, the number of lymphoid follicles and obliterative vasculitis, or fibrosis types. The eosinophil count was higher in the IgG4-RD group than in the PC-iMCD group (p = 0.004). The IgG4/IgG-positive cell ratio was significantly higher in the IgG4-RD group (p < 0.001). The IgA-positive cell count and IL-6 expression intensity were higher in the PC-iMCD group than in the IgG4-RD group (p < 0.001). Based on these findings, we proposed a new diagnostic approach to differentiate lung lesions of PC-iMCD and IgG4-RD. Our approach can be utilized to stratify patients with suspected lung-dominant PC-iMCD to identify candidates for strong immunosuppressive treatment, including IL-6 blockade, at an early stage.

Synchronous Unicentric Castleman Disease and Inflammatory Hepatocellular Adenoma: a Case Report

Systemic symptoms such as fever and fatigue are non-specific manifestations spanning from inflammation to neoplasia. Here we report the case of a 34 year-old man who presented with systemic symptoms for four months. CT-scan and MRI revealed a 3.4 cm arterialized hepatic lesion and a 7 cm paraduodenal mass. Surgical resection of both lesions and histological examination revealed an inflammatory hepatocellular adenoma and a unicentric plasma cell type of Castleman disease. Moreover, a diffuse AA amyloid deposition in the liver was observed. Resection of both lesions was associated with an improvement of the symptoms. To our knowledge, this is the first report of a synchronous presentation of a unicentric plasma cell type of Castleman disease, inflammatory hepatocellular adenoma and AA amyloidosis.

Abdominal localization of unicentric form of Castleman disease: A case report

Introduction. Castleman disease is a rare disease of the unknown etiology, occuring in two clinical forms: unicentric or multicentric. It is characterized by the hyperplasia of lymph glands. In literature the four pathohistological forms were described: hyaline vascular type, plasma cell type, mixed type and a recently recognized plasmablastic type. The most frequent changes are localized in the mediastinum, while the abdominal localization is with significatly rare occurrence, and that was the motive for presentation of this case. Case report. In a 41-year old male magnetic resonance (MR) enterography showed a change in the ileocecal area without the presence of subjective symptoms of digestive tract and without loss of body mass. Due to the suspicion of stromal tumor, surgical intervention was indicated. Pathohistological findings showed Castleman lymphadenopathia reactiva mesenterii (plasma cell type) which was in the unicentric form. There were present only anaemia and the increased value of sedimentation from the laboratory analyses. Conclusion. Abdominal localization of unicentric plasma cell form occurs rarely and the surgical method of treatment presents the golden standard as it was shown in the presented case.

Multicentric Hyaline-Vascular Castleman Disease: A Case Report

Castleman disease is a rare lymphoproliferative disorder. Contrary to its closest differential of lymphoma, the disease tends to run a benign course. However the multicentric variety, which is usually of the plasma cell type, has a more sinister prognosis. It is commonly associated with HIV infection. We report a case of a 65 year old man presenting with recurrent episodes of swelling of the whole body and diarrhoea for 3 years. Physical examination revealed generalized lymphadenopathy with hepato-splenomegaly and ascites. Lymph node biopsy revealed histopathological changes consistent with hyaline-vascular type of Castleman disease (multicentric). He was HIV negative. Patient was treated with high dose corticosteroids and discharged with follow up advice. DOI: http://dx.doi.org/10.3329/jom.v16i1.22402 J MEDICINE 2015; 16 : 48-50