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Chapter 22 - Histiocytic Lesions

from Section V - Histiocytic Neoplasm and Miscellaneous Bone Marrow Diseases

Published online by Cambridge University Press:  25 November 2023

Silvia Tse Bunting
Affiliation:
Cleveland Clinic Florida Weston
Xiayuan Liang
Affiliation:
University of Colorado
Michele E. Paessler
Affiliation:
University of Pennsylvania School of Medicine
Satheesh Chonat
Affiliation:
Emory University, Atlanta
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Summary

Histiocytic disorders consist of many rare related and unrelated wide-ranging proliferations of cells supposedly derived from or sharing common immunophenotypes with macrophages (such as hemophagocytic lymphohistiocytosis) and dendritic cells (such as juvenile xanthogranulomas). These lesions can involve virtually any organ, resulting in an assortment of clinical presentations and prognostic outcomes that vary from localized incidental self-limiting lesions to multisystemic potentially fatal conditions that require chemotherapy or other aggressive treatments. Recent advances in our understanding of histiocytic lesions have shed new light on the pathophysiology of histiocytic disorders, revealing that many distinct entities have overlapping mutations of BRAF or other genes in the MAPK pathway [1]. In 2016, the Histiocyte Society proposed a revised classification system in which histiocytic disorders are sorted into five groups (summarized in Table 22.1) based on clinical, radiologic, histopathologic, immunophenotypic, and genetic/molecular characteristics [2].

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Publisher: Cambridge University Press
Print publication year: 2023

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References

Rollins, BJ. Biology and genomics of LCH and related disorders. In Abla, O and Janka, G, eds. Histiocytic disorders. New York, Springer; 2018:5371.CrossRefGoogle Scholar
Emile, JF, Abla, O, Fraitag, S, Horne, A, Haroche, J, Donadieu, J, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016; 127(22): 2672–81.CrossRefGoogle ScholarPubMed
Foucar, E, Rosai, J, Dorfman, R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Semin Diagn Pathol. 1990; 7(1): 1973.Google ScholarPubMed
Henter, JI, Horne, A, Aricó, M, Egeler, RM, Filipovich, AH, Imashuku, S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48(2): 124–31.CrossRefGoogle ScholarPubMed

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