Rosai Dorfman disease and peripheral T-cell lymphoma

Tiwari, Vivek; Pareek, Ananya; Ghori, Hameeduzzafar; Ahirwar, Manish Kumar

doi:10.4103/jpgm.jpgm_356_18

Cited by 3 publications

(2 citation statements)

References 6 publications

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“…Anti-BRAF V600E immunohistochemistry was strongly positive in both RDD and LCH, and BRAF p.V600E mutation was detected on peripheral blood mononuclear cells. 20 Richardson et al 19 demonstrated a single somatic pathogenic mutation in exon 12 of the BRAF gene (p.486-491del) Types of lymphoma include Hodgkin's lymphoma-classic (8) 25 26 35 and nodular lymphocyte predominant (8) 35 36 and non-Hodgkin's lymphoma-follicular (2), 25 T cell (3), [29][30][31] small lymphocytic lymphoma (2), 35 37 Mantle cell (1), 38 marginal zone lymphoma (3), 35 39 40 diffuse large B cell lymphoma (4), 27 28 41 42 mycosis fungoides (1) 32 and other (large cell immunoblastic and small non-cleaved, 2). 33 34 Types of leukaemia include acute myeloid leukaemia (1) 43 and acute lymphoblastic leukaemia (2).…”

Section: Aetiopathogenesismentioning

confidence: 99%

“…4 Sporadic RDD is the most common form and includes the classic nodal form, extranodal RDD, neoplasia-associated RDD and immune disease-associated RDD. 4 Neoplasms associated with RDD include lymphomas, [25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42] leukaemias, [43][44][45] malignant histiocytoses, 46 47 LCH [48][49][50][51] and ECD 52 (see table 2). Systemic lupus erythematosus, idiopathic juvenile arthritis, autoimmune haemolytic anaemia and HIV are among the immune disorders that are associated with RDD.…”

Section: Classificationmentioning

confidence: 99%

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Rosai-Dorfman disease: an overview

2020

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ContextRosai-Dorfman disease is an uncommon histiocytic disorder most frequently presenting as bilateral cervical lymphadenopathy in children and young adults. Extranodal disease occurs in a significant proportion of patients. It has been recently classified as part of the ‘R group’ of histiocytoses by the Histiocyte Society in 2016. Cutaneous Rosai-Dorfman disease is regarded as a separate disease entity that falls into the ‘C group’ of histiocytoses according to this classification system. The pathogenesis was previously poorly understood; however, recent evidence demonstrating clonality in a subset of cases raises the possibility of a neoplastic process. A possible association with IgG4-related disease remains controversial.ObjectivesTo provide a comprehensive review of Rosai-Dorfman disease, including nodal, extranodal and cutaneous forms, with a particular emphasis on new insights into the possible clonal nature of the disease; to discuss the recently revised classification of the histiocytoses by the Histiocyte Society; and to summarise the findings from the literature regarding the controversial association with IgG4-related disease.Data sourcesThis review is based on published peer-reviewed English literature.ConclusionsClassic Rosai-Dorfman disease, which may be sporadic or familial, is considered a separate entity from cutaneous disease, which is reflected in the revised classification of histiocytoses. An increase in IgG4-positive plasma cells may be seen in Rosai-Dorfman disease. This finding in isolation is of limited significance and should be interpreted with caution. Studies investigating the molecular profile of the disease show that in at least a subset of cases the disease is a clonal process. The classification of Rosai-Dorfman disease is therefore likely to change as our understanding of the aetiopathogenesis evolves.

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Section: Aetiopathogenesismentioning

confidence: 99%

Section: Classificationmentioning

confidence: 99%

Rosai-Dorfman disease: an overview

2020

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Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease

Kiruthiga

Younes

Natkunam

2022

American Journal of Clinical Pathology

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Objectives Rosai-Dorfman disease (RDD) is a rare disorder characterized by the accumulation of large S100 protein-positive histiocytes that typically exhibit emperipolesis. The recently reported expression of Oct-2 in RDD histiocytes led us to explore whether PU.1, a transcription factor that is required for monocyte and B-cell development, could similarly function as a diagnostic marker in RDD. Methods We evaluated the expression of PU.1 and Oct-2 using immunohistochemistry in 19 patients diagnosed with RDD involving nodal, extranodal, and cutaneous sites. Results Both PU.1 and Oct-2 were positive in all cases studied, with a strong intensity of staining in 84% of cases in which more than 50% of the lesional cells were positive. In three patients, both markers showed weak to moderate intensity of staining. Two patients had concomitant RDD and Langerhans cell histiocytosis in which PU.1 stained both types of histiocytes while Oct-2 stained only the RDD component. Conclusions PU.1 emerged as a robust marker with crisp nuclear staining in RDD histiocytes as well as in engulfed inflammatory cells. Strong coexpression of PU.1 and Oct-2 is a useful diagnostic marker in differentiating histiocytic/dendritic cell proliferations.

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How I Diagnose Rosai-Dorfman Disease

Ravindran

Rech

2023

American Journal of Clinical Pathology

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Objectives 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. Methods We discuss the diagnosis of RDD using 2 illustrative cases, interpretative challenges, and a diagnostic algorithm. Results 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. Conclusions 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.

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Rosai Dorfman disease and peripheral T-cell lymphoma

Cited by 3 publications

References 6 publications

Rosai-Dorfman disease: an overview

Rosai-Dorfman disease: an overview

Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease

How I Diagnose Rosai-Dorfman Disease

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