Results 1 to 10 of about 1,120 (144)

Kaposiform Lymphangiomatosis Effectively Treated With MEK Inhibition [PDF]

EMBO Molecular Medicine, 2020
Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly primarily affecting the mediastinum with high mortality rate. We present a patient with KLA and significant disease burden harboring a somatic point mutation in the Casitas B lineage lymphoma
Michael E. March, Jessica B Foster
exaly   +6 more sources

Kaposiform Lymphangiomatosis in a Male Adolescent: A Clinical Challenge and the Role of Genetics [PDF]

Journal of Investigative Medicine High Impact Case Reports, 2023
Kaposiform lymphangiomatosis (KLA) is a rare and aggressive generalized lymphatic anomaly (GLA), with distinctive clinical, radiology, morphologic, and genetic features.
Jorge Lima, Maria Bom-Sucesso, Joana Pereira-Nunes   +2 more
exaly   +6 more sources

Challenging Diagnosis of Kaposiform Lymphangiomatosis: Unveiling a Rare Primary Lymphatic Disorder [PDF]

Radiology Case Reports
Kaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly associated with substantial morbidity and mortality. It features diffuse and multifocal malformed lymphatic channels, often leading to diagnostic difficulties due to its uncommon ...
Rafael Ospino, Claire Brookmeyer
exaly   +7 more sources

Sirolimus in the treatment of kaposiform lymphangiomatosis [PDF]

Orphanet Journal of Rare Diseases, 2021
Background Kaposiform lymphangiomatosis (KLA), which is a new subtype of generalized lymphatic anomaly, is a rare disease with a poor prognosis. Currently, there is no standard treatment due to the poor understanding of KLA.
Jiangyuan Zhou, Kaiying Yang, Siyuan Chen, Yi Ji   +3 more
doaj   +6 more sources

Refractory kaposiform lymphangiomatosis relieved by splenectomy [PDF]

Frontiers in Pediatrics, 2023
IntroductionKaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly with a poor prognosis. There is no standard treatment, and drug therapies are the most common therapeutic method.
Yuru Lan, Jiangyuan Zhou, Tong Qiu, Xue Gong, Yi Ji   +4 more
doaj   +6 more sources

Interventional embolization for hemorrhagic pleural effusion in an infant with kaposiform lymphangiomatosis: a case report [PDF]

Frontiers in Medicine
BackgroundKaposiform lymphangiomatosis (KLA) is a rare, aggressive lymphatic anomaly that frequently involves the thorax and may be complicated by hemorrhagic pleural or pericardial effusion, thrombocytopenia, and coagulopathy.
Jiayi Li, Rui Wang, Rui Wang, Chuan Liu, Chuan Liu, Lingjun Liu, Lingjun Liu   +6 more
doaj   +6 more sources

Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences [PDF]

Orphanet Journal of Rare Diseases, 2019
Background Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax.
Yi Ji, Siyuan Chen, Suhua Peng, Chunchao Xia, Li Li   +4 more
doaj   +5 more sources

Long-term low-dose sirolimus therapy and successful discontinuation in an adult with kaposiform lymphangiomatosis and disseminated intravascular coagulation: a case report [PDF]

Frontiers in Medicine
Kaposiform lymphangiomatosis is a rare, aggressive lymphatic anomaly frequently complicated by disseminated intravascular coagulation. We report a 23-year-old male with a confirmed NRAS p.Q61R mutation who was treated with low-dose sirolimus (1 mg/day ...
Junya Liu, Whenshuai Xu, Whenshuai Xu, Qingwei Jiang, Kai-Feng Xu, Kai-Feng Xu   +5 more
doaj   +2 more sources

Kaposiform lymphangiomatosis—the effects of long-term treatment with sirolimus: case series study and review of the literature [PDF]

Frontiers in Medicine
IntroductionKaposiform lymphangiomatosis (KLA) is an ultrarare disease characterized by abnormal lymphatic vessel proliferation due to hyperactivation of the Rat sarcoma virus (RAS) signaling pathway, resulting in multifocal lymphatic malformations (LMs)
Magdalena Bilny-Paluch, Piotr Piekarczyk, Katarzyna Błasińska, Małgorzata Szołkowska, Mateusz Polaczek, Elżbieta Radzikowska   +5 more
doaj   +2 more sources

Detection of NRAS mutation in cell-free DNA biological fluids from patients with kaposiform lymphangiomatosis [PDF]

Orphanet Journal of Rare Diseases, 2019
Background Kaposiform lymphangiomatosis (KLA) has recently been distinguished as a novel subtype of generalized lymphatic anomaly (GLA) with foci of spindle endothelial cells.
Michio Ozeki, Yoko Aoki, Akifumi Nozawa, Shiho Yasue, Saori Endo, Yumiko Hori, Kentaro Matsuoka, Tetsuya Niihori, Ryo Funayama, Matsuyuki Shirota, Keiko Nakayama, Toshiyuki Fukao   +11 more
doaj   +2 more sources