title: Determination of plasma heparin level improves identification of systemic mast cell activation disease.
abstract: Diagnosis of mast cell activation disease (MCAD), i.e. systemic mastocytosis (SM) and idiopathic systemic mast cell activation syndrome (MCAS), usually requires demonstration of increased mast cell (MC) mediator release. Since only a few MC mediators are currently established as biomarkers of MCAD, the sensitivity of plasma heparin level (pHL) as an indicator of increased MC activation was compared with that of serum tryptase, chromogranin A and urinary N-methylhistamine levels in 257 MCAD patients. Basal pHL had a sensitivity of 41% in MCAS patients and 27% in SM patients. Non-pharmacologic stimulation of MC degranulation by obstruction of venous flow for 10 minutes increased the sensitivity of pHL in MCAS patients to 59% and in SM patients to 47%. In MCAS patients tryptase, chromogranin A, and N-methylhistamine levels exhibited low sensitivities (10%, 12%, and 22%, respectively), whereas sensitivities for SM were higher (73%, 63%, and 43%, respectively). Taken together, these data suggest pHL appears more sensitive than the other mediators for detecting systemic MC activity in patients with MCAS. The simple, brief venous occlusion test appears to be a useful indicator of the presence of pathologically irritable MCs, at least in the obstructed compartment of the body.
authors.affiliation: Institute of Exp. Haematology & Transfusion Medicine, University Hospital of Bonn, Bonn, Germany.
authors.forename: Milda
authors.initials: M
authors.lastname: Vysniauskaite
authors.affiliation: Institute of Exp. Haematology & Transfusion Medicine, University Hospital of Bonn, Bonn, Germany.
authors.forename: Hans-Jörg
authors.initials: HJ
authors.lastname: Hertfelder
authors.affiliation: Institute of Exp. Haematology & Transfusion Medicine, University Hospital of Bonn, Bonn, Germany.
authors.forename: Johannes
authors.initials: J
authors.lastname: Oldenburg
authors.affiliation: Department of Internal Medicine, St. Franziskus Hospital, Eitorf, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Dreßen
authors.affiliation: Department of Oncology, Hematology and Palliative Care, County Hospital, Waldbröl, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Brettner
authors.affiliation: Community Hospital St. Elisabeth, Bonn, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Homann
authors.affiliation: Institute of Human Genetics, University Hospital of Bonn, Bonn, Germany.
authors.forename: Gerhard J
authors.initials: GJ
authors.lastname: Molderings
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000925:Anticoagulants / Q000493:pharmacokinetics*; D015415:Biomarkers; D053379:Chromogranin A / Q000097:blood; D005260:Female; D006493:Heparin / Q000493:pharmacokinetics*; D006801:Humans; D008297:Male; D008407:Mast Cells / Q000187:drug effects / Q000276:immunology* / Q000378:metabolism; D008415:Mastocytosis / Q000097:blood* / Q000175:diagnosis / Q000276:immunology*; D008761:Methylhistamines / Q000097:blood; D008875:Middle Aged; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D053802:Tryptases / Q000097:blood; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D015415:Biomarkers; D053379:Chromogranin A; D008761:Methylhistamines; D006493:Heparin; D053802:Tryptases; C048140:N-methylhistamine