%0 Journal Article
%T Prevalence of hypersensitivity reactions in various forms of mastocytosis: A pilot study of 2485 adult patients with mastocytosis collected in the ECNM registry.
%A Niedoszytko M
%A Gorska A
%A Brockow K
%A Bonadonna P
%A Lange M
%A Kluin-Nelemans H
%A Oude-Elberink H
%A Sabato V
%A Shoumariyeh K
%A von Bubnoff D
%A Müller S
%A Illerhaus A
%A Doubek M
%A Angelova-Fischer I
%A Hermine O
%A Arock M
%A Elena C
%A Malcovati L
%A Yavuz AS
%A Schug TD
%A Fortina AB
%A Judit V
%A Gotlib J
%A Panse J
%A Vucinic V
%A Reiter A
%A Schwaab J
%A Triggiani M
%A Mattsson M
%A Breynaert C
%A Romantowski J
%A Zanotti R
%A Olivieri E
%A Zink A
%A van de Ven A
%A Stefan A
%A Barete S
%A Caroppo F
%A Perkins C
%A Kennedy V
%A Christen D
%A Jawhar M
%A Luebke J
%A Parente R
%A Levedahl K
%A Hadzijusufovic E
%A Hartmann K
%A Nedoszytko B
%A Sperr WR
%A Valent P
%J Allergy
%V 0
%N 0
%D 2024 Apr 23
%M 38651829
%F 14.71
%R 10.1111/all.16132
%X <B>BACKGROUND: </B>Hypersensitivity reactions (HR) are common in mastocytosis. However, little is known about triggers and risk factors. The registry of the European Competence Network on Mastocytosis (ECNM) enables reliable studies in a larger cohort of mastocytosis patients. We assessed prevalence, triggers and risk factors of HR in adults with mastocytosis in the ECNM registry.<BR><B>METHODS: </B>Data were collected in 27 ECNM centers. We analyzed potential triggers (Hymenoptera venoms, food, drug, inhalant and others) and risk factors at diagnosis and during follow-up. The study group consisted of 2485 adults with mastocytosis, 1379 women (55.5%) and 1106 men (44.5%). Median age was 48.2 years (range 18-91 years).<BR><B>RESULTS: </B>Nine hundred and forty eight patients (38.1%) reported one or more HR`. Most common triggers were Hymenoptera venoms in cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), whereas in advanced SM (advSM), most common elicitors were drugs, including nonsteroidal anti-inflammatory agents and penicillin. In multivariate analyses, tryptase level < 90 ng/mL, <15% infiltration by mast cells in bone marrow biopsy-sections, and diagnosis of ISM were identified as independent risk factors for HR. For drug-induced HR, prominent risk factors were advSM and high tryptase levels. New reactions were observed in 4.8% of all patients during 4 years follow-up.<BR><B>CONCLUSIONS: </B>HR are mainly triggered by Hymenoptera venoms in patients with CM and ISM and by drugs in patients with advSM. Tryptase levels <90 ng/mL, mast cell bone marrow infiltration <15%, and WHO category ISM are predictors of HR. New HR occur in 4.8% of all patients within 4 years.