title: Chymase expressing bone marrow mast cells in mastocytosis and myelodysplastic syndromes: an immunohistochemical and morphometric study.
abstract: BACKGROUND
  Two cell specific neutral proteases, tryptase and chymase, are produced by human mast cells (MC). Tryptase is constitutively expressed by all MC, whereas chymase is found only in an MC subset. Very little is known about chymase expression in MC proliferative disorders (mastocytosis).
  
  
  OBJECTIVE
  Routinely processed, formalin fixed, and paraffin wax embedded bone marrow trephine biopsy specimens obtained from patients with various subtypes of mastocytosis (n = 47) and myelodysplastic syndromes (MDS; n = 28) were immunostained with antibodies against chymase and tryptase. Normal/reactive bone marrow specimens with intact haemopoiesis (n = 31) served as controls. The numbers of chymase expressing (C+) and of tryptase expressing (T+) MC were assessed morphometrically using a computer assisted video camera system.
  
  
  RESULTS
  In normal/reactive bone marrow, the numbers of C+ MC (median, 8/mm(2); maximum, 159/mm(2)) were in the same range as those of T+ MC (median, 4/mm(2); maximum, 167/mm(2)). Because normal MC express both chymase and tryptase, these findings indicate that the common phenotype of bone marrow MC in normal/reactive states is MC(TC) (MC expressing both tryptase and chymase). In contrast, in MDS and mastocytosis, the bone marrow exhibited far more T+ MC than C+ MC in almost all cases.
  
  
  CONCLUSIONS
  According to these findings, the predominant MC type in the bone marrow in neoplastic states such as MDS and mastocytosis is MC(T) (MC expressing only tryptase). Although the pathophysiological basis of this apparent lack of chymase expression in most neoplastic MC in mastocytosis and MC involved in MDS remains unknown, this study has produced further evidence of the superior value of antitryptase antibodies in the diagnosis of mastocytosis.
authors.affiliation: Institute of Pathology, University of Lübeck, Ratzeburger Allee 160, D-23538 Lübeck, Germany. horny@patho.mu-luebeck.de
authors.forename: H-P
authors.initials: HP
authors.lastname: Horny
authors.forename: A
authors.initials: A
authors.lastname: Greschniok
authors.forename: J-H
authors.initials: JH
authors.lastname: Jordan
authors.forename: D M
authors.initials: DM
authors.lastname: Menke
authors.forename: P
authors.initials: P
authors.lastname: Valent
mesh_terms: D001854:Bone Marrow Cells / Q000201:enzymology*; D002452:Cell Count; D053818:Chymases; D006801:Humans; D007124:Immunoenzyme Techniques; D008407:Mast Cells / Q000201:enzymology*; D008415:Mastocytosis / Q000201:enzymology* / Q000473:pathology; D009190:Myelodysplastic Syndromes / Q000201:enzymology* / Q000473:pathology; D012697:Serine Endopeptidases / Q000378:metabolism*; D053802:Tryptases
chemical_list: D012697:Serine Endopeptidases; D053818:Chymases; D053802:Tryptases