We are excited to share that the Kaposiform Hemangioendothelioma (KHE) Patient Pathway, developed by the VASCA Working Group of VASCERN, is now available. This document is designed specifically for healthcare professionals managing patients with KHE, offering practical, expert-backed guidance to support accurate diagnosis, effective treatment, and long-term care.
What Is Kaposiform Hemangioendothelioma (KHE)?
Kaposiform Hemangioendothelioma, or KHE, is a rare and aggressive vascular tumour that primarily affects infants and young children. This condition can manifest as reddish-purple lesions on the skin or develop deeper in tissues such as the chest, abdomen, or retroperitoneal space. Unlike common infantile haemangiomas, KHE does not regress on its own and may lead to serious complications.
One of the most critical complications associated with KHE is Kasabach-Merritt Phenomenon (KMP). KMP is a life-threatening condition marked by severe thrombocytopenia (a dangerously low platelet count) and coagulopathy (clotting abnormalities). This complication significantly increases the risk of bleeding and must be treated promptly with specialised, multidisciplinary care.
The new pathway offers healthcare professionals a detailed, evidence-based framework to address the complexities of KHE and improve patient outcomes, including managing complications like KMP.
What does the KHE Patient Pathway include?
The KHE Patient Pathway serves as a comprehensive guide for clinicians, covering key areas such as:
- Diagnostic Work-Up
- Treatment Strategies
- Long-Term Management and Follow-Up
- Patient Support
Why is this pathway important?
KHE is a challenging condition to manage due to its rarity, complexity, and potential for life-threatening complications. The KHE Patient Pathway ensures that healthcare professionals have a clear, step-by-step guide to navigate these challenges effectively. By integrating the latest clinical insights and expert consensus, the pathway supports consistent, high-quality care for KHE patients across Europe.
The pathway also highlights the importance of multidisciplinary collaboration, recognising that successful outcomes often depend on input from haematologists, radiologists, surgeons, and other specialists in vascular anomalies.
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