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• W2334443568 abstract "Castleman's disease is a rare lymphoid disorder. It comprises two pathological entities. These are the hyaline-vascular type, which is usually localized (uni-centeric), and the plasma cell type which is usually multicenteric and rather aggressive. Castleman's disease has been described thrice after kidney transplantation. A 62-year-old Caucasian male with a history of kidney transplant presented high fever, night sweats, dyspnoea, coughing, weakness, hepatosplenomegaly, peripheral lymphadenopathy and symptoms of renal failure within 6 years of the transplantation. Laboratory findings revealed anaemia (haemoglobin = 4.6 mmol/l), thrombocytopenia (101 × 109/L), normal WBC total and differential count, renal function impairment (creatinine = 218 μmol/l), hypoalbuminemia (24 g/l), and elevated CRP (133 mg/l). Diagnostic assays for HIV-1 were performed with repeatedly negative results. No acute CMV or EBV infection was detected (by serology and PCR analysis). The clinical diagnosis at this stage was post-transplant lymphoproliferative disorder, therefore dosage of cyclosporin immunosuppression was decreased. CT-scans revealed hepatosplenomegaly and enlarged abdominal lymph nodes. A bone marrow biopsy showed an active haematopoiesis in all cell lineages without any abnormalities. Histological examination of an axillary lymph node biopsy showed a multifocal proliferation of spindle cells. The diagnosis of Kaposi's sarcoma was confirmed by positive staining of these spindle cells with antibodies against factor VIII-related antigen (not shown) and HHV-8. The remainder of the lymph node showed partly involuted germinal centres, surrounded by small lymphocytes. A number of these lymphocytes also stained positive for HHV-8, as seen in multicentric Castleman's disease. The clinical syndrome and the histological findings were compatible with KS and MCD caused by HHV-8 infection in a long-term immunocompromised patient. HHV-8 DNA load in serum or plasma was determined by real-time quantitative PCR (15 × 105 copies/ml). His condition deteriorated rapidly and he died despite therapy. The unicentric Castelman's disease, histopathologically described as the hyaline-vascular subtype, is more common, it is not associated with HHV8-infection, and can mostly be treated effectively with radiotherapy or surgery. The multicentric form is not well understood: it is an atypical lymphoproliferative disorder of a plasma cell type, and is related to immune dysfunction. Patients with MCD often develop malignancies like Kaposi's sarcoma and non-Hodgkin's lymphoma. Clinically, HHV8-positive MCD is more aggressive, and has a poorer prognosis with a median survival of less than 30 months. MCD, whether or not HHV8-associated, is generally treated with combination chemotherapy and steroids." @default.
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• W2334443568 date "2012-11-01" @default.
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• W2334443568 title "Concurrent and Fatal HHV-8 Positive Multicentric Castlemanʼs Disease and Kaposiʼs Sarcoma in a HIV Negative Kidney Transplant Recipient" @default.
• W2334443568 doi "https://doi.org/10.1097/00007890-201211271-00713" @default.
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