Email this article Kaposiform lymphangiomatosis with Kasabach–Merritt phenomenon
- Authors: Khachatryan L.A.1, Novichkova G.A.1, Vasilieva M.S.1, Kletskaya I.S.1, Scherbakov A.P.1, Maschan A.A.1
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Affiliations:
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
- Issue: Vol 22, No 2 (2023)
- Pages: 142-151
- Section: CLINICAL OBSERVATIONS
- Submitted: 28.03.2023
- Accepted: 24.05.2023
- Published: 08.07.2025
- URL: https://hemoncim.com/jour/article/view/730
- DOI: https://doi.org/10.24287/1726-1708-2023-22-2-142-151
- ID: 730
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Abstract
Kaposiform lymphangiomatosis (KLA) is an aggressive lymphatic anomaly associated with bone involvement, serositis occurring at various sites, the development of Kasabach–Merritt phenomenon, and frequent infectious complications. The International Society for the Study of Vascular Anomalies classifies KLA as a subtype of generalized lymphatic anomaly. The mTOR-inhibitor rapamycin in combination with symptomatic treatment is the most common specific treatment. However, there are no standard approaches to the management of KLA. Even with modern diagnostic tools and combination therapy, the 5-year survival rate is 51 %, and the average life expectancy is 2.75 years. This article presents a classic case of KLA associated with Kasabach–Merritt phenomenon that was successfully managed with rapamycin and a liposomal form of doxorubicin as specific therapy. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.
About the authors
L. A. Khachatryan
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Author for correspondence.
Email: lili.2510@yandex.ru
ORCID iD: 0000-0001-7265-0414
Lili A. Khachatryan, Cand. Med. Sci., Head of Unit
Hematology / Oncology Isolation Unit
117997
1 Samory Mashela St.
Moscow
Russian FederationG. A. Novichkova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-2322-5734
Moscow
Russian FederationM. S. Vasilieva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Moscow
Russian FederationI. S. Kletskaya
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Moscow
Russian FederationA. P. Scherbakov
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Moscow
Russian FederationA. A. Maschan
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-0016-6698
Moscow
Russian FederationReferences
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