Rosai-Dorfman-Destombes disease in adults: A single center experience Journal Article
| Authors: | Leung, E.; Pryma, C.; Murphy, S.; Harrison, R.; Peterson, E.; Tsang, P. W. K.; Varghese, J.; You, X.; Slack, G. W.; Skinnider, B. F.; Ng, T.; Young, S.; Burrell, S.; Stubbins, R.; Lim, H.; Carruthers, M.; Dutz, J.; Diamond, E. L.; Chen, L. Y. C. |
| Article Title: | Rosai-Dorfman-Destombes disease in adults: A single center experience |
| Abstract: | Recent advances in Rosai-Dorfman-Destombes disease (RDD), notably molecular testing, targeted therapy, and PET-CT imaging, hold promise for better recognition and improved outcomes. This study presents patients diagnosed and treated in a “real world” setting, where navigating limited resources must be considered. This retrospective single-center review includes 15 adult patients diagnosed with RDD at Vancouver General Hospital between November 2015 and October 2023. The cohort comprised five males and ten females with a median age 53 years (range 19–80 years). All 15 patients had extra-nodal disease; 11 patients exclusively had extra-nodal disease, and four patients also had lymph node involvement. Seven patients had tissue next-generation sequencing, identifying MAP2K1 mutations in four cases and a KRAS p.K117N mutation in one case that was treated with targeted therapy using trametinib. PET-CT was used for disease staging in four cases. Six patients with refractory disease tolerated lenalidomide and dexamethasone without significant toxicity; three patients achieved complete response, and three had partial response. This study highlights RDD's diverse extra-nodal manifestations. Lenalidomide combined with dexamethasone is an effective and well-tolerated treatment option for select patients, especially those with refractory disease. Broad utilization of NGS and PET-CT can positively influence management decisions. © The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2024. |
| Keywords: | adult; clinical article; treatment response; aged; aged, 80 and over; middle aged; retrospective studies; young adult; gene mutation; genetics; lenalidomide; histopathology; rituximab; nuclear magnetic resonance imaging; follow up; c reactive protein; gene; computer assisted tomography; thrombocytopenia; dexamethasone; retrospective study; dyspnea; interleukin 6; pleura effusion; histiocytosis; kras; toxicity; protein electrophoresis; sirolimus; immunoglobulin g4; cladribine; pleura biopsy; kras gene; cereblon; heart atrium flutter; tocilizumab; trametinib; high throughput sequencing; map2k1 gene; next-generation sequencing; very elderly; humans; human; male; female; article; sinus histiocytosis; rosai-dorfman disease; histiocytosis, sinus; positron emission tomography-computed tomography; positron emission tomography computed tomography; abdominal paracentesis; immunoglobulin g4 related disease; sclerosing mesenteritis |
| Journal Title: | Annals of Hematology |
| Volume: | 103 |
| Issue: | 11 |
| ISSN: | 0939-5555 |
| Publisher: | Springer |
| Date Published: | 2024-11-01 |
| Start Page: | 4467 |
| End Page: | 4476 |
| Language: | English |
| DOI: | 10.1007/s00277-024-06019-w |
| PUBMED: | 39331156 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Article -- MSK Cancer Center Support Grant (P30 CA008748) acknowledged in PDF -- MSK corresponding author is Eli Diamond -- Source: Scopus |
