title: Systemic mast cell disease without associated hematologic disorder: a combined retrospective and prospective study.
abstract: OBJECTIVE
  To study clinicopathologic correlations and identify prognostically important variables in patients with systemic mast cell disease (SMCD) who have no associated hematologic disorders.
  
  
  METHODS
  We identified 40 adult patients with SMCD without associated hematologic disorders. Clinical, laboratory, and bone marrow (BM) histologic findings at the time of referral were evaluated (November 1980-February 2001) for possible correlations with a history of aggressive systemic mastocytosis (retrospectively analyzed) as well as survival (prospectively analyzed).
  
  
  RESULTS
  The median follow-up time from diagnosis was 108 months and from BM examination was 24 months. A history of aggressive systemic mastocytosis correlated significantly with increased BM mast cell (MC) content, unfavorable MC infiltration pattern, BM eosinophilia, and elevated serum alkaline phosphatase (SAP) level, but not with BM angiogenesis, reticulin fibrosis, or levels of MC mediators. Of these factors, increased BM MC content and elevated SAP level were also associated with shortened survival from time of referral.
  
  
  CONCLUSIONS
  This study suggests that the BM MC burden, BM eosinophilia, and SAP level are prognostically important in SMCD without associated hematologic disorders. In contrast, BM angiogenesis, reticulin fibrosis, and levels of MC mediators showed no prognostic relevance.
authors.affiliation: Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Jin-Young
authors.initials: JY
authors.lastname: Baek
authors.forename: Chin-Yang
authors.initials: CY
authors.lastname: Li
authors.forename: Joseph H
authors.initials: JH
authors.lastname: Butterfield
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology*; D005260:Female; D006402:Hematologic Diseases / Q000150:complications / Q000401:mortality* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008407:Mast Cells / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000150:complications / Q000401:mortality* / Q000473:pathology*; D008875:Middle Aged; D009389:Neovascularization, Pathologic / Q000473:pathology*; D011336:Probability; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D018570:Risk Assessment; D018709:Statistics, Nonparametric; D016019:Survival Analysis