Kaposiform lymphangiomatosis: diagnosis, pathogenesis, and treatment

CG McDaniel, DM Adams, KE Steele… - Pediatric blood & …, 2023 - Wiley Online Library
Kaposiform lymphangiomatosis (KLA) is a life‐threatening rare disease that can cause
substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis …

Sirolimus plus prednisolone vs sirolimus monotherapy for kaposiform hemangioendothelioma: a randomized clinical trial

Y Ji, S Chen, J Zhou, K Yang, X Zhang… - Blood, The Journal …, 2022 - ashpublications.org
Abstract The Kasabach-Merritt phenomenon (KMP) in kaposiform hemangioendothelioma
(KHE) is characterized by life-threatening thrombocytopenia and consumptive coagulopathy …

Medical Therapeutics for the Treatment of Vascular Anomalies: Part 3

K Ricci - Oral and Maxillofacial Surgery Clinics, 2024 - oralmaxsurgery.theclinics.com
Optimal management of complex vascular anomalies aims to not only treat acute issues,
such as infection or effusion, but also prevent potential future complications and improve …

Treatment of severe Kaposiform lymphangiomatosis positive for NRAS mutation by MEK inhibition

G Chowers, G Abebe-Campino, H Golan, A Vivante… - Pediatric …, 2023 - nature.com
Background Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly
involving most commonly the mediastinum, lung, skin and bones with few effective …

Kaposiform lymphangiomatosis: pathologic aspects in 43 patients

AR Perez-Atayde, L Debelenko… - The American Journal …, 2022 - journals.lww.com
Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with
distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document …

Complex lymphatic anomalies: report on a patient registry using the latest diagnostic guidelines

TAA Andreoti, S Berg, A Holm, M Angerer… - … research and biology, 2023 - liebertpub.com
Background: Generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD),
kaposiform lymphangiomatosis (KLA), and central conducting lymphatic anomaly (CCLA) …

Cellular variant of kaposiform lymphangiomatosis: a report of three cases, expanding the morphologic and molecular genetic spectrum of this rare entity

W Allen-Rhoades, A Al-Ibraheemi, M Kohorst… - Human Pathology, 2022 - Elsevier
Kaposiform lymphangiomatosis (KLA) is a very rare form of generalized lymphatic anomaly,
consisting of a diffuse proliferation of abnormal, dilated lymphatics, and small fascicles of …

Sirolimus for kaposiform hemangioendothelioma: Potential mechanisms of action and resistance

T Qiu, S Xiang, J Zhou, M Yang, Y Lan… - … Journal of Cancer, 2025 - Wiley Online Library
Kaposiform hemangioendotheliomas (KHEs) are vascular tumors that are considered
borderline or locally aggressive and may lead to lethal outcomes. Traditional therapies, such …

[HTML][HTML] Toxic effects of sirolimus and everolimus on the development and behavior of zebrafish embryos

Z Zhang, T Qiu, J Zhou, X Gong, K Yang… - Biomedicine & …, 2023 - Elsevier
Sirolimus and everolimus have been widely used in children. These mammalian target of
rapamycin (mTOR) inhibitors have shown excellent efficacy not only in organ transplant …

Targeted therapies for vascular malformations

G Kane, I Fernandez-Pineda - Frontiers in Medicine, 2024 - frontiersin.org
Targeted medical therapies for the treatment of vascular malformations is an exciting and
evolving area of research. As the identification of specific causative genetic mutations …