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What is Familial Rosai-Dorfman Disease (RDD)- Not Otherwise Specified

Chapter 11
Principles and Approaches to Diagnostic Bone Marrow Examination
A rare variant of RDD characterized by a familial pattern of occurrence, has an autosomal recessive inheritance, and is associated with mutations in the SLC29A3 gene on chromosome 10q22. It is part of the ‘ R group ’ of histiocytoses, which includes familial RDD, sporadic RDD, and other miscellaneous non-cutaneous, non-Langerhans cell histiocytoses. The disorder may be associated with IgG4-related disease.
Published in Chapter:
Other Neoplasms of Myeloid Origin: Histiocytic/Dendritic-Cell and Mast Cell Neoplasms
Copyright: © 2024 |Pages: 44
DOI: 10.4018/978-1-6684-5818-1.ch011
Abstract
Other rare neoplasms may arise from myeloid progenitors, including Histiocytic/ Dendritic Neoplasms and mast cell neoplasms. The Classification of both categories in the 5th edition of WHO has some modifications with newly introduced entities. Histiocytic neoplasms exhibit diverse somatic oncogenic alterations. Their clinical presentation ranges from incidental findings to critical illness with severe organ dysfunction. Each subtype has distinct morphologic and immunophenotypic features. However, some overlap exists between the different subtypes. Mastocytosis involves abnormal mast cells accumulating in various organs. It develops as a clonal expansion of mast cells derived by constitutive activation of the KIT receptor. The clinical course varies from asymptomatic to diffuse systemic involvement with comorbidities mainly related to mediators' release.
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