Results 1 to 10 of about 307 (89)

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   +4 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   +5 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

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
Jessica B Foster, Dong Li, Michael E March, Sarah E Sheppard, Denise M Adams, Hakon Hakonarson, Yoav Dori   +6 more
doaj   +4 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.
Joana Pereira-Nunes MD, Miguel Madureira MD, Alexandra Dinis MD, Helena Barroca MD, Jorge Lima Msc, Maria do Bom-Sucesso MD   +5 more
doaj   +4 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, BS, Claire Brookmeyer, MD
doaj   +4 more sources

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment. [PDF]

Pediatr Blood Cancer, 2023
AbstractKaposiform lymphangiomatosis (KLA) is a life‐threatening rare disease that can cause substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis often occurs long after initial symptoms, and there are few centers in the world with the expertise to diagnose and care for patients with the disease.
McDaniel CG, Adams DM, Steele KE, Hammill AM, Merrow AC, Crane JL, Smith CL, Kozakewich HPW, Le Cras TD.   +8 more
europepmc   +3 more sources

Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients. [PDF]

Am J Surg Pathol, 2022
Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document the pathology in 43 patients evaluated by the Boston Children’s Hospital Vascular Anomalies Center from 1999 to 2020.
Perez-Atayde AR, Debelenko L, Al-Ibraheemi A, Eng W, Ruiz-Gutierrez M, O'Hare M, Croteau SE, Trenor CC, Boyer D, Balkin DM, Barclay SF, Hsi Dickie B, Liang MG, Chaudry G, Alomari AI, Mulliken JB, Adams DM, Kurek KC, Fishman SJ, Kozakewich HPW.   +19 more
europepmc   +3 more sources

Kaposiform lymphangiomatosis presenting with a Group A Streptococcus pericardial effusion. [PDF]

BMJ Case Rep, 2022
A 4-year-old child was transferred to the paediatric intensive care unit with acute respiratory failure following 4 days of fever, nausea and vomiting. Chest X-ray on admission had an enlarged cardiac silhouette and transthoracic echo confirmed a large pericardial effusion.
Koerner T, Patel MD, Pai V, Indramohan G.   +3 more
europepmc   +4 more sources

Treatment of severe Kaposiform lymphangiomatosis positive for NRAS mutation by MEK inhibition. [PDF]

Pediatr Res, 2023
Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly involving most commonly the mediastinum, lung, skin and bones with few effective treatments. In recent years, RAS-MAPK pathway mutations were shown to underlie the pathogenesis of several complex lymphatic anomalies.
Chowers G, Abebe-Campino G, Golan H, Vivante A, Greenberger S, Soudack M, Barkai G, Fox-Fisher I, Li D, March M, Battig MR, Hakonarson H, Adams D, Dori Y, Dagan A.   +14 more
europepmc   +3 more sources