TY - JOUR
T1 - Tryptase and histamine metabolites as diagnostic indicators of indolent systemic mastocytosis without skin lesions
AU - van Doormaal, J. J.
AU - van der Veer, E.
AU - van Voorst Vader , P. C.
AU - Kluin, P. M.
AU - Mulder, A. B.
AU - van der Heide, S.
AU - Arends, S.
AU - Kluin-Nelemans, J. C.
AU - Oude Elberink, J. N. G.
AU - de Monchy, J. G. R.
PY - 2012/5
Y1 - 2012/5
N2 - Background: Risk indicators of indolent systemic mastocytosis (ISM) in adults with clinical suspicion of ISM without accompanying skin lesions [urticaria pigmentosa (UP)] are lacking. This study aimed at creating a decision tree using clinical characteristics, serum tryptase, and the urinary histamine metabolites methylimidazole acetic acid (MIMA) and methylhistamine (MH) to select patients for bone marrow investigations to diagnose ISM.Methods: Retrospective data analysis of all adults, in whom bone marrow investigations were performed to diagnose ISM, was carried out.Results: In total, 142 patients were included. SM was absent in all 44 patients with tryptase = 10 mu g/l and in 18 of 52 patients (35%) with tryptase > 20 mu g/l. Above 43 mu g/l, all patients had ISM (n = 11). Male gender, insect venom anaphylaxis as presenting symptom, tryptase, MIMA, and MH were independent ISM predictors. If tryptase was >= 10 mu g/l, the diagnostic accuracy of MIMA and MH was high (areas under the ROC curve 0.92).Conclusions: In suspected patients without UP, the ISM risk is very low (if present at all) if tryptase is = 10 mu g/l, this risk depends on MIMA and MH, being low if these are normal, but high if these are elevated. Male gender and insect venom anaphylaxis are additional risk indicators. We recommend refraining from bone marrow examinations in suspected patients without UP if tryptase is 20 mu g/l.
AB - Background: Risk indicators of indolent systemic mastocytosis (ISM) in adults with clinical suspicion of ISM without accompanying skin lesions [urticaria pigmentosa (UP)] are lacking. This study aimed at creating a decision tree using clinical characteristics, serum tryptase, and the urinary histamine metabolites methylimidazole acetic acid (MIMA) and methylhistamine (MH) to select patients for bone marrow investigations to diagnose ISM.Methods: Retrospective data analysis of all adults, in whom bone marrow investigations were performed to diagnose ISM, was carried out.Results: In total, 142 patients were included. SM was absent in all 44 patients with tryptase = 10 mu g/l and in 18 of 52 patients (35%) with tryptase > 20 mu g/l. Above 43 mu g/l, all patients had ISM (n = 11). Male gender, insect venom anaphylaxis as presenting symptom, tryptase, MIMA, and MH were independent ISM predictors. If tryptase was >= 10 mu g/l, the diagnostic accuracy of MIMA and MH was high (areas under the ROC curve 0.92).Conclusions: In suspected patients without UP, the ISM risk is very low (if present at all) if tryptase is = 10 mu g/l, this risk depends on MIMA and MH, being low if these are normal, but high if these are elevated. Male gender and insect venom anaphylaxis are additional risk indicators. We recommend refraining from bone marrow examinations in suspected patients without UP if tryptase is 20 mu g/l.
KW - bone marrow
KW - histamine metabolites
KW - indolent systemic mastocytosis
KW - tryptase
KW - NITROGEN-PHOSPHORUS DETECTION
KW - BLOOD MONONUCLEAR-CELLS
KW - N-TAU-METHYLHISTAMINE
KW - C-KIT MUTATION
KW - URINE
KW - EXCRETION
KW - DISORDERS
KW - MARKERS
U2 - 10.1111/j.1398-9995.2012.02809.x
DO - 10.1111/j.1398-9995.2012.02809.x
M3 - Article
SN - 0105-4538
VL - 67
SP - 683
EP - 690
JO - Allergy
JF - Allergy
IS - 5
ER -