Cases of Childhood Mastocytosis: A Single Center Experience

Author:

Çevik Seda1ORCID,Altas Ugur1ORCID,Çiçek Fatih2ORCID,Altaş Zeynep Meva3ORCID,Çetemen Ayşen1ORCID,Özkars Mehmet Yaşar1ORCID

Affiliation:

1. Umraniye Training and Research Hospital

2. Kartal Dr. Lütfi Kırdar Şehir Hastanesi

3. Ümraniye Toplum Sağlığı Merkezi

Abstract

Objective: Cutaneous mastocytosis, primarily affecting children, is confined to the skin and generally carries a good prognosis. In our study, we aimed to evaluate the clinical findings, laboratory values and treatment-related data of 10 patients who were followed up with a diagnosis of mastocytosis in our clinic between 2014 and 2022. Methods: Age, gender, family history, clinical findings, type of lesions, laboratory values and treatment-related data of the patients were analyzed within the scope of the study. Skin biopsy was taken from clinically suspected patients and the diagnosis was made with histopathologic confirmation. Histopathologic diagnosis was made by demonstration of mast cells showing metachromasia with toluidine blue in full-thickness skin biopsy. Results: The median age at presentation was 10.0 months (min-max: 1.0-117.0). While rash and pruritus were the most common complaints seen in all patients; erythema was seen in 9 (90%) patients. The most common rash type was maculopapular. One (10.0%) patient had nodules and mastocytoma. When the laboratory findings of the patients were evaluated, no patient had thrombocytopenia or leukopenia. One patient had anemia. The median value of total IgE values was 65.0 IU/ml (8.0-1719.0). Conclusion: In our study, all patients had symptoms of rash and pruritus. The most common lesion type in our study was maculopapular rash (UP type) seen in 4 patients (40%). Nodules and mastocytoma (NM type) were seen in 1 patient (10%). In our study covering an eight-year period, all of our patients had cutaneous mastocytosis and none of them had systemic involvement.

Publisher

New Trends in Medicine Sciences

Reference23 articles.

1. 1. Valent P. Mastocytosis: a paradigmatic example of a rare disease with complex biology and pathology. Am J Cancer Res. 2013; 3(2):159-72.

2. 2. Akin C, Valent P. Diagnostic criteria and classification of mastocytosis in 2014. Immunol Allergy Clin North Am. 2014; 34:207-18.

3. 3. Horny HP, Akin C, Metcalfe DD, et al. Mastocytosis (mast cell disease). World Health Organization (WHO). Classification of tumors: pathology and genetics. In: Tumours of haematopoietic and lymphoid tissues. Swerdlow SH, Campo E, Harris NL (eds). IARC Press, Lyon 2008;54-63.

4. 4. Sandru F, Petca RC, Costescu M, et al. Cutaneous Mastocytosis in Childhood-Update from the Literature. J Clin Med. 2021; 10(7):1474.

5. 5. Macri A, Cook C. Urticaria Pigmentosa. In: StatPearls. Treasure Island (FL): StatPearls Publishing; November 13, 2023.

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3