Affordable Access

Access to the full text

Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management

Authors
  • Ji, Yi1
  • Chen, Siyuan2
  • Xia, Chuncao1
  • Zhou, Jiangyuan1
  • Jiang, Xian1
  • Xu, Xuewen1
  • Yang, Kaiying1
  • Zhang, Xuepeng2
  • Kong, Feiteng3
  • Lu, Guoyan4
  • Zhang, Yongbo5
  • 1 West China Hospital of Sichuan University, Chengdu, 610041, China , Chengdu (China)
  • 2 West China Hospital of Sichuan University, #37# Guo-Xue-Xiang, Chengdu, 610041, China , Chengdu (China)
  • 3 Sichuan Women and Children’s Hospital, Chengdu, 610045, China , Chengdu (China)
  • 4 West China Second University Hospital, Sichuan University, Chengdu, 610041, China , Chengdu (China)
  • 5 Chengdu Women and Children’s Central Hospital, Chengdu, 610031, China , Chengdu (China)
Type
Published Article
Journal
Orphanet Journal of Rare Diseases
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Nov 07, 2020
Volume
15
Issue
1
Identifiers
DOI: 10.1186/s13023-020-01595-2
Source
Springer Nature
Keywords
License
Green

Abstract

ObjectivesThere are no cohort studies of chronic lymphedema in patients with kaposiform hemangioendothelioma (KHE). We sought to characterize the incidence, clinical features, risk factors and management of chronic lymphedema in patients with KHE.MethodsWe conducted a multicenter retrospective analysis of patients who had a minimum of 3 years of follow-up after the onset of KHE and/or Kasabach–Merritt phenomenon (KMP). Clinical features were reviewed to determine the possible cause of chronic lymphedema. The degree of lymphedema, risk factors and management strategies were analyzed.ResultsAmong the 118 patients, chronic lymphedema was confirmed by lymphoscintigraphy 1 year after the onset of KHE and/or KMP in 13 patients. In 8 patients with lymphedema, extremity swelling was evident in the presence of KHE and/or KMP. In all patients with lymphedema, a unilateral extremity was affected, along with ipsilateral KHE. Most (84.6%) patients reported moderate lymphedema. Lymphedema was more common in patients with larger (≥ 10 cm) and mixed lesions involving the extremities (P < 0.01). A history of KMP and sirolimus treatment were not predictors of lymphedema (P > 0.05). Overall, 76.9% of patients received sirolimus treatment after referral, including 53.8% who presented extremity swelling before referral. Seven (53.8%) patients received compression therapy. Five (38.5%) patients reported lymphedema-associated decreased range of motion at the last follow-up.ConclusionsChronic lymphedema is a common sequela of KHE and can occur independently of KMP and sirolimus treatment. Patients with large and mixed KHE involving extremities should be closely monitored for this disabling complication.

Report this publication

Statistics

Seen <100 times