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Castleman's disease and related disorders
1Department of Laboratory Medicine and Pathology, University of MinnesotaMedical School, Minneapolis 55455.
Insights
Castleman disease (CD) encompasses three disorders: localized hyaline-vascular, localized plasma cell, and multicentric CD. These conditions represent distinct B cell lymphoproliferative disorders affecting lymph nodes, with multicentric CD considered a systemic B cell lymphoproliferation.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Castleman disease (CD) is an eponym for three distinct clinicopathologic disorders.
- Localized CD presents as either hyaline-vascular (HV) type (asymptomatic mass, abnormal lymphoid tissue) or plasma cell (PC) type (systemic inflammation, B cell hyperreactivity).
- Multicentric CD is a systemic B cell lymphoproliferation with mixed histology, peripheral lymphadenopathy, and multisystem involvement.
Purpose of the Study:
- To discuss the three disorders classified under the Castleman disease eponym.
- To differentiate the clinical and histological features of localized HV, localized PC, and multicentric CD.
- To contextualize localized PC and multicentric CD within the spectrum of idiopathic plasma cell disorders.
Main Methods:
- Review and discussion of existing literature and clinicopathologic data on Castleman disease variants.
- Comparative analysis of histological findings, clinical presentations, and disease behavior.
- Classification of CD subtypes within a framework of B cell lymphoproliferative disorders and lymphocyte traffic.
Main Results:
- Localized HV CD is characterized by hypervascularity and abnormal lymphoid tissue, often presenting as a localized mass.
- Localized PC CD involves plasma cell infiltration and systemic inflammation, appearing as a localized chronic reaction.
- Multicentric CD is a systemic disorder with mixed histology, systemic symptoms, and potential for clonal B cell outgrowth, related to POEMS syndrome.
Conclusions:
- Castleman disease encompasses a spectrum of B cell disorders ranging from hyperplasia to dysplasia.
- Localized PC CD is viewed as a hyperplastic disorder, while multicentric CD is considered dysplastic.
- These conditions are linked to idiopathic plasma cell disorders and involve peripheral-node-bound B cells.
Abstract:
Three disorders that bear the eponym of Castleman's disease (CD) are discussed. The localized CD of hyaline-vascular (HV) type features an architecturally abnormal, hypervascular lymphoid tissue with burned-out germinal centers, and presents as an asymptomatic, slowly growing mass. It may represent a lymphoid hyperplasia associated with excessive angiogenesis. The localized CD of plasma cell (PC) type, instead, features an architecturally recognizable lymph node with solid sheets of PCs and presents with systemic manifestations of inflammation and B cell hyperreactivity. It appears as a localized chronic reaction to unknown antigens. "Multicentric" CD indicates a clinicopathologic entity characterized by the histology of CD of "mixed" type, a predominantly lymphadenopathic presentation consistently involving peripheral nodes, manifestations of multisystem involvement, and an idiopathic nature. It is best considered as a systemic B cell lymphoproliferation, which probably arises in a setting of immunoregulatory deficit, and may result in the outgrowth of clonal B cell populations. An attempt is presented to place the latter two forms of CD in the larger perspective of idiopathic PC disorders, by taking into account both the nature of the diseases (hyperplastic, dysplastic, neoplastic) and the lymphocyte traffic system that is involved (bone marrow-bound, mucosa-associated or peripheral-node-bound). In such a scheme, localized CD, PC type, and multicentric CD appear as a hyperplastic and a dysplastic disorder, respectively, of peripheral-node-bound B cells, related to, and often associated with, primary nodal plasmacytoma and osteosclerotic myeloma (so-called POEMS, Takatsuki's or Crow-Fukase's syndrome).