Rosai-Dorfman Disease



Rosai-Dorfman Disease


Faisal Alseraye, MD

L. Jeffrey Medeiros, MD










Touch imprint of lymph node involved by Rosai-Dorfman disease. Numerous histiocytes with emperipolesis and small lymphocytes are present. The histiocytes show emperipolesis, particularly prominent in some cells image.






Lymph node involved by Rosai-Dorfman disease (RDD). S100 stain highlights the cytoplasm of RDD histiocytes. S100 negatively outlines intracytoplasmic lymphocytes image in cells with emperipolesis.


TERMINOLOGY


Abbreviations



  • Rosai-Dorfman disease (RDD)


Synonyms



  • Sinus histiocytosis with massive lymphadenopathy (SHML)


  • Histiocytose lipidique ganglionnaire pseudotumorale de Destombes


Definitions



  • Benign proliferation of histiocytes with characteristic cytologic features



    • Histiocytes show emperipolesis (engulfment of lymphocytes)


    • Histiocytes express S100


ETIOLOGY/PATHOGENESIS


Unknown



  • RDD has been reported in identical twins or families suggesting genetic predisposition


  • Associated with autoimmune lymphoproliferative syndrome


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare; worldwide geographic distribution


  • Age



    • Wide range



      • Newborn to ˜ 75 years; more common in children


  • Gender



    • M:F = 3:2 (1.5)


  • Ethnicity



    • All races affected


Site



  • Lymph nodes


  • Extranodal sites in ˜ 40% of patients



    • Head and neck region common



      • Upper respiratory tract, skin


    • Other common sites



      • Skin, soft tissues, gastrointestinal tract


      • Bones, breast, dura


      • Almost any site can be involved


Presentation



  • Lymphadenopathy, often without any symptoms



    • Usually localized


    • Cervical lymph nodes most often involved


    • Often bilateral with massive enlargement


  • B symptoms are uncommon but can occur



    • Fever, night sweats can precede lymphadenopathy


  • Laboratory abnormalities in subset of patients



    • Polyclonal hypergammaglobulinemia common


    • Blood lymphocytes with low CD4 to CD8 ratio


    • Hemolytic anemia


Treatment



  • In most patients, RDD regresses spontaneously



    • No specific therapy needed


    • RDD often persists for months before regression


    • Rarely RDD can persist for years before regression


  • Rare subset of patients have aggressive RDD and require therapy



    • Therapies: Steroids, radiation therapy, chemotherapy


    • Surgical excision for patients with obstruction/compression-type symptoms


Prognosis



  • Excellent for most affected patients


  • Rare cases can be clinically aggressive



    • No effective therapy for these rare aggressive cases


    • Fatalities can occur as a result of



      • Accompanying immune dysregulation


      • Mass effect in vital organs



IMAGE FINDINGS


Radiographic Findings



  • Lymphadenopathy


MACROSCOPIC FEATURES


General Features



  • Enlarged lymph nodes: Often massive



    • Often matted with capsular fibrosis


MICROSCOPIC PATHOLOGY


Histologic Features

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Rosai-Dorfman Disease
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