The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience
Introduction: In children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determinati...
Main Authors: | , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Termedia Publishing House
2017-08-01
|
Series: | Advances in Dermatology and Allergology |
Subjects: | |
Online Access: | https://www.termedia.pl/The-role-of-serum-tryptase-in-the-diagnosis-and-monitoring-of-pediatric-mastocytosis-a-single-center-experience,7,30413,1,1.html |
id |
doaj-89489966e4d0444cb090a190f2d69de7 |
---|---|
record_format |
Article |
spelling |
doaj-89489966e4d0444cb090a190f2d69de72020-11-24T22:59:56ZengTermedia Publishing HouseAdvances in Dermatology and Allergology1642-395X2299-00462017-08-0134430631210.5114/ada.2017.6930830413The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experienceMagdalena LangeAgata ZawadzkaStephanie SchrörsJustyna SłomkaHanna Ługowska-UmerBogusław NedoszytkoRoman NowickiIntroduction: In children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determination is recommended in the diagnosis and follow-up. Aim: To compare clinical presentation and the course of mastocytosis in children with normal and clearly elevated bsT levels as well as to assess its usefulness in the diagnosis and monitoring of the course of the disease. Material and methods: A retrospective analysis of 102 medical records of children with mastocytosis diagnosed and followed-up in the Gdansk Mastocytosis Center in 2014 was performed. Results: Maculopapular CM (MPCM) was diagnosed in 91 (89.22%) children, diffuse cutaneous mastocytosis (DCM) in 7 (6.86%) and mastocytoma in 4 (3.92%). The presence of flushing and bullous lesions was more frequent in children with bsT levels > 20 ng/ml in comparison with those who had bsT ≤ 20 ng/ml (p = 0.002 and p = 0.03, respectively). Anaphylaxis occurred in 2 MPCM children with bsT levels in normal ranges. In all of the 3 children with persistently and clearly elevated bsT levels, bone marrow biopsy revealed no mast cells infiltrates corresponding to SM. Conclusions: Although mastocytosis children with clearly elevated bsT levels frequently develop mediator-related symptoms, the occurrence of anaphylaxis in this age group may be difficult to predict. In pediatric cases with suspected SM, the bsT level is one of the crucial parameters considered before the decision on bone marrow biopsy.https://www.termedia.pl/The-role-of-serum-tryptase-in-the-diagnosis-and-monitoring-of-pediatric-mastocytosis-a-single-center-experience,7,30413,1,1.htmlmastocytosis serum tryptase children diagnosis |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Magdalena Lange Agata Zawadzka Stephanie Schrörs Justyna Słomka Hanna Ługowska-Umer Bogusław Nedoszytko Roman Nowicki |
spellingShingle |
Magdalena Lange Agata Zawadzka Stephanie Schrörs Justyna Słomka Hanna Ługowska-Umer Bogusław Nedoszytko Roman Nowicki The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience Advances in Dermatology and Allergology mastocytosis serum tryptase children diagnosis |
author_facet |
Magdalena Lange Agata Zawadzka Stephanie Schrörs Justyna Słomka Hanna Ługowska-Umer Bogusław Nedoszytko Roman Nowicki |
author_sort |
Magdalena Lange |
title |
The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience |
title_short |
The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience |
title_full |
The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience |
title_fullStr |
The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience |
title_full_unstemmed |
The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience |
title_sort |
role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience |
publisher |
Termedia Publishing House |
series |
Advances in Dermatology and Allergology |
issn |
1642-395X 2299-0046 |
publishDate |
2017-08-01 |
description |
Introduction: In children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determination is recommended in the diagnosis and follow-up.
Aim: To compare clinical presentation and the course of mastocytosis in children with normal and clearly elevated bsT levels as well as to assess its usefulness in the diagnosis and monitoring of the course of the disease.
Material and methods: A retrospective analysis of 102 medical records of children with mastocytosis diagnosed and followed-up in the Gdansk Mastocytosis Center in 2014 was performed.
Results: Maculopapular CM (MPCM) was diagnosed in 91 (89.22%) children, diffuse cutaneous mastocytosis (DCM) in 7 (6.86%) and mastocytoma in 4 (3.92%). The presence of flushing and bullous lesions was more frequent in children with bsT levels > 20 ng/ml in comparison with those who had bsT ≤ 20 ng/ml (p = 0.002 and p = 0.03, respectively). Anaphylaxis occurred in 2 MPCM children with bsT levels in normal ranges. In all of the 3 children with persistently and clearly elevated bsT levels, bone marrow biopsy revealed no mast cells infiltrates corresponding to SM.
Conclusions: Although mastocytosis children with clearly elevated bsT levels frequently develop mediator-related symptoms, the occurrence of anaphylaxis in this age group may be difficult to predict. In pediatric cases with suspected SM, the bsT level is one of the crucial parameters considered before the decision on bone marrow biopsy. |
topic |
mastocytosis serum tryptase children diagnosis |
url |
https://www.termedia.pl/The-role-of-serum-tryptase-in-the-diagnosis-and-monitoring-of-pediatric-mastocytosis-a-single-center-experience,7,30413,1,1.html |
work_keys_str_mv |
AT magdalenalange theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT agatazawadzka theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT stephanieschrors theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT justynasłomka theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT hannaługowskaumer theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT bogusławnedoszytko theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT romannowicki theroleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT magdalenalange roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT agatazawadzka roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT stephanieschrors roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT justynasłomka roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT hannaługowskaumer roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT bogusławnedoszytko roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience AT romannowicki roleofserumtryptaseinthediagnosisandmonitoringofpediatricmastocytosisasinglecenterexperience |
_version_ |
1725643281351573504 |