Rosai Dorfman Disease Pathology
Rosai dorfman disease pathology. Rosai-Dorfman Disease S100 The abnormal histiocytes show nuclear expression of S100 whereas the engulfed lymphoid cells and neutrophils do not. Rosai-Dorfman disease RDD described by Rosai and Dorfman Rosai and Dorfman 1969 in 1969 also known as Rosai-Dorfman-Destombes disease and sinus histiocytosis with massive lymphadenopathy is a rare cause of adenopathy which is more prevalent in children and young adults. Usually undergoes spontaneous remission after a protracted course but may recur and rarely involves vital organs causing death.
Rosai-Dorfman-Destombes disease RDD is a rare nonLangerhans cell histiocytosis LCH first described in 1965 by a French pathologist Pierre Paul Louis Lucien Destombes who reported 4 children and young adults with lymphadenopathy and sinus histiocytosis upon histologic analysis. Cutaneous Rosai-Dorfman disease typically occurs in older females and presents in various forms ranging from single papules to multiple nodules and plaques. RosaiDorfman disease RDD is a rare nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features.
May represent extension from lymph node. Isolated Intraocular Rosai-Dorfman Disease. Rosai Dorfman disease sinus histiocytosis.
1Department of Pathology The University of Texas MD Anderson Cancer Center Houston TX 77030 USA. The cytoplasm of the abnormal histiocytic cells is also reactive with S100 causing the nonlabeled emperipolesed cells to stand out on the S100 stain. It has been recently classified as part of the R group of histiocytoses by the Histiocyte Society in 2016.
Rosai Dorfman disease sinus histiocytosis with massive lymphadenopathy SHML is a benign self-limiting disorder that commonly involves the lymph nodes. 2Department of Pathology Städtisches Klinikum Karlsruhe Karlsruhe Germany. 1 Four years later Juan Rosai and Ronald Dorfman analyzed 34 cases of the same entity under the name sinus.
Methods Articles that reviewed tumor biology clinical features pathology and treatment for RDD were identified in a search of the literature for the years 1990 to 2014. Because of its rarity the diagnosis is often not initially considered in the differential. Rosai-Dorfman disease RDD is a rare nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features.
13 This same society has recently reclassified the. Extranodal disease occurs in a significant proportion of patients.
Usually undergoes spontaneous remission after a protracted course but may recur and rarely involves vital organs causing death.
Rosai-Dorfman disease RDD is a rare nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features. Rosai-Dorfman-Destombes disease RDD is a rare nonLangerhans cell histiocytosis LCH first described in 1965 by a French pathologist Pierre Paul Louis Lucien Destombes who reported 4 children and young adults with lymphadenopathy and sinus histiocytosis upon histologic analysis. 2Department of Pathology Städtisches Klinikum Karlsruhe Karlsruhe Germany. Fogt F1 Rüediger T2 Augustin AJ3 Frank DM1 Rosenwald A4 Wellmann A5 Lee V16. View chapter Purchase book. 1Department of Pathology The University of Texas MD Anderson Cancer Center Houston TX 77030 USA. Rosai-Dorfman disease RDD is a rare nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features. It usually involves the cervical lymph nodes and less commonly extranodal sites. That in at least a subset of cases the disease is a clonal process.
1Department of Pathology University of Pennsylvania Philadelphia Pennsylvania USA. Rosai-Dorfman-Destombes disease RDD is a rare histiocytic disorder described by Destombes in 1965 and later by Rosai and Dorfman in 1969 as sinus histiocytosis with massive lymphadenopathy and previously classified by the Working Group of the Histiocyte Society of 1987 as a non-Langerhans cell LC histiocytosis. Extranodal disease occurs in a significant proportion of patients. Fogt F1 Rüediger T2 Augustin AJ3 Frank DM1 Rosenwald A4 Wellmann A5 Lee V16. Rosai-Dorfman disease RDD described by Rosai and Dorfman Rosai and Dorfman 1969 in 1969 also known as Rosai-Dorfman-Destombes disease and sinus histiocytosis with massive lymphadenopathy is a rare cause of adenopathy which is more prevalent in children and young adults. Isolated Intraocular Rosai-Dorfman Disease. Usually undergoes spontaneous remission after a protracted course but may recur and rarely involves vital organs causing death.
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