{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T03:50:45Z","timestamp":1793332245284,"version":"4.5.0"},"reference-count":46,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2023,5,11]],"date-time":"2023-05-11T00:00:00Z","timestamp":1683763200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,7,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Rosai-Dorfman disease (RDD) is one of 3 major types of histiocytosis, along with Erdheim-Chester disease and Langerhans cell histiocytosis. While historically, RDD was considered a benign self-limited condition, current data show MAPK\/ERK pathway mutations in 30% to 50% of cases, indicative of a clonal process. Rosai-Dorfman disease was incorporated as a histiocytic neoplasm in the fifth edition of the World Health Organization classification of hematopoietic tumors and the International Consensus Classification.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We discuss the diagnosis of RDD using 2 illustrative cases, interpretative challenges, and a diagnostic algorithm.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Rosai-Dorfman disease involves nodal and extranodal sites, including skin, sinuses, salivary gland, orbit, central nervous system, kidney, and bone. In a subset, RDD can coexist with other neoplasms (lymphomas, other histiocytosis) or autoimmune disease. Morphologically, RDD histiocytes are characterized by enlarged round to oval nuclei, distinct nucleoli, and voluminous cytoplasm with engulfment of inflammatory cells (emperipolesis). By immunohistochemistry, they express CD68, CD163 (majority), S100, OCT2, and cyclin D1. Appropriate use of ancillary studies is important to support the diagnosis of RDD while excluding other histiocytic neoplasms and reactive histiocytic proliferations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Management of RDD is dependent on the extent of organ involvement and clinical symptoms. In patients who require therapy, next-generation sequencing is recommended to identify MAPK\/ERK pathway mutations for targeted therapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ajcp\/aqad047","type":"journal-article","created":{"date-parts":[[2023,5,11]],"date-time":"2023-05-11T12:42:44Z","timestamp":1683808964000},"page":"1-10","source":"Crossref","is-referenced-by-count":54,"title":["How I Diagnose Rosai-Dorfman Disease"],"prefix":"10.1093","volume":"160","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4503-440X","authenticated-orcid":false,"given":"Aishwarya","family":"Ravindran","sequence":"first","affiliation":[{"name":"Department of Pathology, Division of Laboratory Medicine-Hematopathology, University of Alabama at Birmingham , Birmingham, AL , US"},{"name":"Department of Laboratory Medicine and Pathology, Division of Hematopathology, Mayo Clinic , Rochester, MN , US"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen L","family":"Rech","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Division of Hematopathology, Mayo Clinic , Rochester, MN , US"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,5,11]]},"reference":[{"key":"2023070513453783700_CIT0001","first-page":"1169","article-title":"Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali (4 cases) [in French]","volume":"58","author":"Destombes","year":"1965","journal-title":"Bull Soc Pathol Exot Filiales"},{"key":"2023070513453783700_CIT0002","first-page":"63","article-title":"Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity","volume":"87","author":"Rosai","year":"1969","journal-title":"Arch Pathol"},{"key":"2023070513453783700_CIT0003","first-page":"19","article-title":"Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review 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