Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder.
OBJECTIVE
Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Immunophenotypically the histiocytes of SHML express intensively the S100 protein and in addition a panel of macrophage-associated antigens. Their exact relationship to either monocytes/macrophages or immune accessory dendritic cells is, however, still controversial.


RESULTS
In this report recurrent nodal and extranodal manifestations of SHML of a 70-year-old patient were analysed by differential phenotyping using a panel of monoclonal and polyclonal antibodies to macrophage and immune accessory dendritic cell related antigens and by applying nonradioactive in-situ hybridization.


CONCLUSIONS
We conclude that stimulation of monocytes/macrophages via macrophage colony stimulating factor (M-CSF) leading to immune suppressive macrophages represents a main mechanism for the pathogenesis of SHML. The study further provides evidence for the monocyte/macrophage but not dendritic cell differentiation of SHML histiocytes.
Middel
P
P
Department of Pathology, Georg August University of Göttingen, Germany. pmiddel@med.uni-goettingen.de
Hemmerlein
B
B
Fayyazi
A
A
Kaboth
U
U
Radzun
H J
HJ
D015415:Biomarkers / Q000032:analysis; D003713:Dendritic Cells / Q000378:metabolism / Q000473:pathology; D006644:Histiocytes / Q000378:metabolism / Q000473:pathology*; D015618:Histiocytosis, Sinus / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping; D017403:In Situ Hybridization; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology*; D016173:Macrophage Colony-Stimulating Factor / Q000378:metabolism*; D008264:Macrophages / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009000:Monocytes / Q000378:metabolism / Q000473:pathology
D015415:Biomarkers; D016173:Macrophage Colony-Stimulating Factor