Related Experiment Video
Updated: Feb 17, 2026

Identifying Dysregulated Genes Induced by Kaposi's Sarcoma-associated Herpesvirus KSHV
Published on: September 14, 2010
Castleman Disease and Rosai-Dorfman Disease
Miguel A Piris1, Elena Aguirregoicoa1, Santiago Montes-Moreno2
1Pathology Service, Fundación Jiménez Díaz, CIBERONC, Avenida Reyes Católicos, 2, 28040 Madrid, Spain.
Insights
Castleman Disease (CD) and Rosai-Dorfmann Disease (RDD) are distinct lymphoproliferative and histiocytic disorders, respectively. While CD presents heterogeneously, RDD involves specific histiocyte infiltration and may represent a neoplastic process.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Castleman Disease (CD) is a heterogeneous lymphoproliferative disorder with overlapping features.
- Rosai-Dorfmann Disease (RDD) is a histiocytic proliferative disorder characterized by specific cellular infiltrates.
- Both conditions require careful histopathological differentiation.
Purpose of the Study:
- To delineate the distinct features of Castleman Disease (CD) and Rosai-Dorfmann Disease (RDD).
- To highlight the diagnostic criteria and potential underlying mechanisms of CD and RDD.
Main Methods:
- Review of clinical and histopathological characteristics of CD and RDD.
- Discussion of diagnostic markers such as S100 and CD1a for RDD.
- Exploration of molecular findings suggesting neoplastic potential in RDD.
Main Results:
- CD encompasses Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD, and Multicentric CD, each with unique morphological and clinical aspects.
- HVCD involves abnormal germinal centers and follicular dendritic cell changes.
- Multicentric CD is associated with proinflammatory hypercytokinemia and florid presentation.
- RDD diagnosis relies on S100-positive, CD1a-negative histiocytes, plasma cell aggregates, and emperipolesis.
- Molecular data suggest RDD may be a low-grade neoplastic process in some cases.
Conclusions:
- CD and RDD are distinct entities with specific diagnostic features.
- Understanding these differences is crucial for accurate diagnosis and management.
- Further research into RDD's etiology may reveal neoplastic origins.
Abstract:
This chapter describes the main features of two different diseases, Castleman Disease (CD) and Rosai-Dorfman Disease (RDD). Castleman disease (CD) is a clinical and histopathologically heterogeneous lymphoproliferative disorder that encompasses at least three distinct entities with some common overlapping morphological features: Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD and Multicentric CD. The most important feature of HVCD is the presence of abnormal germinal centers with hyaline-vascular transformation, sometimes showing multiple germinal centers within a single reactive lymphoid follicle, this outlining HVCD as a disorder of follicular dendritic cells. Unicentric and multicentric CD are, in contrast, lymphoproliferative lesions. Proinflammatory hypercytokinemia is an essential feature of multicentric CD, distinguished by a florid clinical presentation. Rosai-Dorfmann Disease is a histiocytic proliferative disorder diagnosed by the presence of tissue infiltration by S100-positive CD1a-negative histiocytes and plasma cell aggregates, often with Russell bodies. A typical, though not specific, characteristic of the disease is emperipolesis. Initially considered to be an inflammatory/reactive condition, molecular studies suggest that at least some cases of RDD could be considered as a low-grade histiocytic neoplastic process.
More Related Videos
10:35A Blood-based Test for the Detection of ROS1 and RET Fusion Transcripts from Circulating Ribonucleic Acid Using Digital Polymerase Chain Reaction
Published on: April 5, 2018
07:17Author Spotlight: Advancing the Detection of Low-Frequency Mutations in Cancer Tissues
Published on: August 23, 2024
Related Concept Videos
Rous Sarcoma Virus (RSV) and Cancer
RSV is a retrovirus that contains two copies of a plus-strand RNA genome. Its genome consists of four main open...
Cardiomyopathy I: Introduction and Classification
Abnormal Proliferation
Cardiomyopathy IV: Restrictive Cardiomyopathy
mTOR Signaling and Cancer Progression
The mTOR pathway or the...
Rheumatic Heart Disease I: Introduction