Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005;129(1):3-17

Department of Medicine, Division of Infectious Disease, University of Washington School of Medicine, and The Program in Infectious Disease, Fred Hutchinson Cancer Research Center, Seattle, WA 98122, USA.
British Journal of Haematology (Impact Factor: 4.71). 05/2005; 129(1):3-17. DOI: 10.1111/j.1365-2141.2004.05311.x
Source: PubMed


Fifty years ago, Dr Benjamin Castleman first described the unusual lymphoproliferative disorder that now bears his name. Over the subsequent decades, astute clinical and pathologic observations coupled with clever molecular biologic research have increased our understanding of the aetiology of Castleman disease (CD). This article proposes three broad CD variants based on both distinctive histopathology and clinical behaviour. The pivotal roles of infection with human herpesvirus 8 and interleukin-6 production in the development of CD are emphasized. Finally, the natural history of CD and the myriad of therapeutic options are reviewed in the context of a unified model of CD pathophysiology, and continued areas of uncertainty are discussed.

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    • "Clinical and laboratory data were available from diagnosis and during treatment for all 114 cases. Clinical diagnoses of CD and complications were established following generally accepted guidelines (Casper, 2005; Fajgenbaum et al, 2014). Of the 114 patients, 105 underwent computerized tomography (CT) examination of involved organs/regions or B-type ultrasonography (USG-B) examination of superficial lymph nodes (including cervical, axillary, inguinal regions and abdominal and pelvic cavities). "
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    ABSTRACT: This study retrospectively collected the clinical and laboratory data of 114 patients with Castleman disease (CD) from a single medical centre. Clinical classification identified 62 patients (54·4%) with unicentric Castleman disease and 52 (45·6%) with multi-centric Castleman disease. Pathological classification revealed 68 cases (59·6%) of hyaline vascular variant, 16 (14·1%) mixed cellular variant (Mix) and 30 (26·3%) plasmacytic variant. Clinical complications occurred in 69 CD patients, including 37 cases of paraneoplastic pemphigus (PNP) and 25 cases with renal complications. Haematological involvement, pleural effusion and/or ascites and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) were also found. Univariate analysis showed that presence of clinical complications and PNP were both risk factors relating to CD patient survival. Prognostic factors showing P < 0·15 in univariate analysis and those with clinical significance were subjected to multivariate analysis using a Cox regression model. PNP presence and age over 40 years both significantly adversely affected survival. Thus, only presence of PNP was identified as an independent unfavourable survival risk factor in both univariate and multivariate analyses. Overall, the present data provide a panoramic description of CD cases and emphasize that the presence of PNP is an adverse prognostic factor. © 2015 John Wiley & Sons Ltd.
    British Journal of Haematology 03/2015; 169(6). DOI:10.1111/bjh.13378 · 4.71 Impact Factor
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    • "The patient has maintained a good clinical course since the surgery (decrease in brain natriuretic peptide from 1390 pg/mL to 614 pg/mL). MCD is a rare systemic lymphoproliferative disorder characterized by abnormal proliferation of polyclonal plasma cells in the lymphoid follicles [1] "

    International journal of cardiology 03/2014; 173(2). DOI:10.1016/j.ijcard.2014.03.054 · 4.04 Impact Factor
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    • "According to a recently published study, there are no differences in the surgical outcomes of deep and superficial Castleman’s disease as long as the resection is complete [27]. The prognosis and outcome of the multicentric type depend on many factors, like progression rate, infections and comorbidities, and tend to be poorer [2,4,6]. There are no clear data about how long follow-up care should be conducted. "
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    ABSTRACT: Castleman's disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman's disease have been reported in the literature. A 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10x9x5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman's disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected. Castleman's disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.
    Journal of Medical Case Reports 03/2014; 8(1):93. DOI:10.1186/1752-1947-8-93
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