Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F21%3A00074373" target="_blank" >RIV/65269705:_____/21:00074373 - isvavai.cz</a>

Result on the web

<a href="https://pure.rug.nl/ws/files/178043597/1_s2.0_S2213219820313544_main.pdf" target="_blank" >https://pure.rug.nl/ws/files/178043597/1_s2.0_S2213219820313544_main.pdf</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.jaip.2020.12.022" target="_blank" >10.1016/j.jaip.2020.12.022</a>

Result language

angličtina

Original language name

Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin

Original language description

BACKGROUND: Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients. OBJECTIVE: To develop a risk score to predict SM in adults with MIS. METHODS: We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 +/- 13.3 years. The median serum tryptase level amounted to 29.3 +/- 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves. RESULTS: In the multivariate model, the tryptase level (P &lt; .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P &lt; .001) were independent predictors of SM (P &lt; .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated. CONCLUSIONS: Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30200 - Clinical medicine

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2021

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Journal of Allergy and Clinical Immunology-In Practice

ISSN

2213-2198

e-ISSN

—

Volume of the periodical

9

Issue of the periodical within the volume

4

Country of publishing house

NL - THE KINGDOM OF THE NETHERLANDS

Number of pages

12

Pages from-to

1705-"+"

UT code for WoS article

000637779800035

EID of the result in the Scopus database

2-s2.0-85099805558