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16 - Castleman’s disease

from Section 3 - Myeloma: clinical entities

Published online by Cambridge University Press:  18 December 2013

By
Karthik Ramasamy
Edited by
Stephen A. Schey ,
Kwee L. Yong ,
Robert Marcus  and
Kenneth C. Anderson
Stephen A. Schey
Affiliation:
Department of Haematology, King’s College Hospital, London
Kwee L. Yong
Affiliation:
Department of Haematology, University College Hospital, London
Robert Marcus
Affiliation:
Department of Haematology, King’s College Hospital, London
Kenneth C. Anderson
Affiliation:
Dana-Farber Cancer Institute, Boston
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Summary

Introduction

Castleman's disease was first described in a case report by Dr. Benjamin Castleman and Towne in 1954[1]. Following this initial description, a case series of mediastinal lymph node hyperplasia which was subsequently coined as Castleman's disease was published[2]. Castleman's disease is a non-clonal lymphoproliferative disorder characterized by angiofollicular lymph node hyperplasia, widespread lymphadenopathy and marked constitutional symptoms in affected patients. Castleman's disease is frequently associated with both human immunodeficiency virus (HIV) and human herpes virus 8 (HHV8) infections. Castleman's disease not infrequently presents with either polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS syndrome), Hodgkin's disease, non-Hodgkin's lymphoma, or Kaposi's sarcoma. Clinical presentation can be more localized, with absence of systemic symptoms termed as unicentric presentation. Patients with multi-centric Castleman's disease present with constitutional symptoms and multiple lymph node areas or organs are involved. Although unicentric presentation is frequent and curable in most patients with surgical resection and/or radiotherapy, management of Multicentric Castleman's disease is challenging. There are two main histological subtypes described in Castleman's disease: hyaline vascular subtype and plasma cell variant. Occasionally mixed patterns can occur. In HIV positive patients, lymph nodes are ubiquitously positive for HHV8 infection. There is significant clinical heterogeneity, and the factors guiding therapy are clinical features, HIV status, performance status, localized or multi-centric presentation and in some cases the type of hematological neoplasm associated with. Castleman's disease is a rare condition and no official incidence or prevalence figures are available. The National Cancer Institute in the USA has assigned an orphan status to Castleman's disease.

Type
Chapter
Information
Myeloma
Pathology, Diagnosis, and Treatment
 , pp. 216 - 224
Publisher: Cambridge University Press
Print publication year: 2013

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References

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