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Potential biomarkers of kaposiform lymphangiomatosis
Pediatric Blood & Cancer, 2019AbstractBackgroundKaposiform lymphangiomatosis (KLA) has recently been distinguished as a novel subtype of generalized lymphatic anomaly (GLA), and is characterized by foci of spindle endothelial cells amid a background of malformed lymphatic channels. The etiology of these diseases remains unknown and diagnosis is confounded by their similar clinical ...
Michio Ozeki +5 more
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Imaging features of kaposiform lymphangiomatosis
Pediatric Radiology, 2016Kaposiform lymphangiomatosis is a rare, aggressive lymphatic disorder. The imaging and presenting features of kaposiform lymphangiomatosis can overlap with those of central conducting lymphatic anomaly and generalized lymphatic anomaly.To analyze the imaging findings of kaposiform lymphangiomatosis disorder and highlight features most suggestive of ...
Pradeep, Goyal +8 more
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Successful treatment of kaposiform lymphangiomatosis with sirolimus
Pediatric Blood & Cancer, 2015Generalized lymphatic anomaly (GLA) is a rare and often fatal congenital lymphatic disorder that also commonly affects bone. Kaposiform lymphangiomatosis (KLA) is a novel subtype of GLA with poor prognosis and no proper treatment guidelines. A 9‐year‐old male with recurrent pleural effusion was clinically diagnosed as KLA.
Zuopeng, Wang +5 more
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Kaposiform lymphangiomatosis: Unifying features of a heterogeneous disorder
Pediatric Blood & Cancer, 2014Kaposiform lymphangiomatosis (KLA) is a rare proliferation of abnormal lymphatic vessels often complicated by pleural/pericardial effusions and a consumptive coagulopathy that may lead to life threatening hemorrhage. Establishing the diagnosis is challenging due to the clinical heterogeneity and variable findings in laboratory values, radiographic ...
Violet M, Fernandes +7 more
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Microscopic Kaposiform Hemangioendothelioma With Extensive Lymphangiomatosis
International Journal of Surgical Pathology, 2012Kaposiform hemangioendothelioma (KHE) is presently classified as a vascular neoplasm of intermediate malignant potential. The clinical course of large, deep-seated tumors is frequently complicated by consumptive coagulopathy and life-threatening hemorrhage, while superficial tumors tend to behave in an indolent manner, with no known reports of distant
Irene C, Low +2 more
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Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis
Child's Nervous System, 2018A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt.
Cristina Barceló-López +5 more
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P23 Stabilization of aggressive disease in NRAS kaposiform lymphangiomatosis with sirolimus
British Journal of Dermatology, 2023Abstract Kaposiform lymphangiomatosis (KLA) is a rare disorder of lymphatic vasculature characterized by infiltrative and multifocal lymphatic abnormalities. The disease course is often complicated by a severe coagulopathy and frequently involves the central conducting lymphatic vessels leading to large-volume chylous pleural/pericardial
Miteshkumar Ramwani +3 more
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Case 343: Kaposiform Lymphangiomatosis
RadiologyHistory A 17-year-old boy presented to the outpatient department with left-sided breast enlargement for 2 years. He also had multiple long bone fractures after trivial trauma over the past 8 years, along with intermittent episodes of streaky hemoptysis.
Divij Agarwal +3 more
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2018
Lymphatic anomalies, including lymphatic malformations and lymphangiomatosis, result from the lymphatic system and have been classified by ISSVA, as described in Chap. 2 of this textbook. When functioning properly, the lymphatic system maintains fluid balance, produces lymphocytes which provide important defense against pathogens, and is crucial to the
Tara L. Wenger, Anne Hing
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Lymphatic anomalies, including lymphatic malformations and lymphangiomatosis, result from the lymphatic system and have been classified by ISSVA, as described in Chap. 2 of this textbook. When functioning properly, the lymphatic system maintains fluid balance, produces lymphocytes which provide important defense against pathogens, and is crucial to the
Tara L. Wenger, Anne Hing
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Pediatric Emergency Care, 2016
Abstract Kaposiform lymphangiomatosis is a generalized lymphatic disorder complicated by consumptive coagulopathy and pericardial and pleural effusions. We present the case of a 13-year-old female adolescent given a diagnosis of a large pleural effusion by point-of-care thoracic ultrasonography, which led to further evaluation and diagnosis ...
Manu S, Raam +2 more
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Abstract Kaposiform lymphangiomatosis is a generalized lymphatic disorder complicated by consumptive coagulopathy and pericardial and pleural effusions. We present the case of a 13-year-old female adolescent given a diagnosis of a large pleural effusion by point-of-care thoracic ultrasonography, which led to further evaluation and diagnosis ...
Manu S, Raam +2 more
openaire +2 more sources