Multifocal lymphangioendotheliomatosis with thrombocytopenia in children: a systematic review and individual patient data analysis

British Journal of Dermatology 2026

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Authors: Gisella Chiara Carpi, Leticia R Campos, Ana Beatriz Nardelli da Silva, Ana Emília Carvalho de Paula, Lilia Maria Lima de Oliveira
DOI: 10.1093/bjd/ljag031
Cited by: 0
References: 5

Abstract

This individual patient meta-analysis of 57 children with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) from 34 studies establishes the condition’s characteristic clinical triad: universal cutaneous involvement (100%), near-universal thrombocytopenia (93%) and gastrointestinal bleeding (92%). We identified novel sex dimorphism, with boys showing significantly higher pulmonary involvement than girls (85% vs. 29%; P = 0.02) and defined a diagnostic immunophenotype (CD31+ CD34+ LYVE-1+ GLUT-1–) that distinguishes MLT from similar vascular anomalies. These findings provide clinicians with evidence-based diagnostic criteria and support emerging mTOR-targeted therapies for this rare, life-threatening paediatric vascular disorder.

Fields of Study & Subjects

Vascular Malformations and Hemangiomas Vascular Tumors and Angiosarcomas Lymphatic System and Diseases

Publication details

Journal / source
British Journal of Dermatology
Volume
194
Issue
5
Pages
967–968
Publication date
29 January 2026
Language
EN