Thoracic duct embolization in kaposiform lymphangiomatosis

Bundy, Jacob J.; Ootaki, Yoshio; McLean, Thomas W.; Hays, Brandon; Miller, Michael E.; Downing, Trevor

doi:10.1016/j.jvsv.2020.03.013

Cited by 6 publications

(6 citation statements)

References 9 publications

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“…The effusions are often hemorrhagic, even in the absence of coagulopathy, and this can be important in the diagnosis of KLA. Patients may present with fatigue, shortness of breath, cough, and fever 1,6 . Skeletal involvement can lead to back, joint, soft tissue, or bone pain.…”

Section: Clinical Presentation and Imagingmentioning

confidence: 99%

“…New therapies have likely improved the prognosis, although updated epidemiological studies are needed to better quantify the prevalence of KLA and survival with available treatments 3 . The diagnosis of KLA is often delayed due to variable presenting symptoms, apprehension concerning obtaining a biopsy due to coagulopathy and risk of lymphatic leakage, and lack of familiarity with this disease 4–6 . Treatment remains challenging as many patients show no or only partial responses, and so there is an urgent need to identify new therapeutic targets and more effective medications 7 .…”

Section: Introductionmentioning

confidence: 99%

“…Treatment remains challenging as many patients show no or only partial responses, and so there is an urgent need to identify new therapeutic targets and more effective medications 7 . Mortality is typically the result of cardiorespiratory failure or coagulopathy 6 …”

Section: Introductionmentioning

confidence: 99%

“…3 The diagnosis of KLA is often delayed due to variable presenting symptoms, apprehension concerning obtaining a biopsy due to coagulopathy and risk of lymphatic leakage, and lack of familiarity with this disease. [4][5][6] Treatment remains challenging as many patients show no or only partial responses, and so there is an urgent need to identify new therapeutic targets and more effective medications. 7 Mortality is typically the result of cardiorespiratory failure or coagulopathy.…”

Section: Introductionmentioning

confidence: 99%

“…7 Mortality is typically the result of cardiorespiratory failure or coagulopathy. 6 The International Society for the Study of Vascular Anomalies (ISSVA) 2018-updated classification defines KLA as a subset of generalized lymphatic anomaly (GLA). However, whether KLA is actually distinct from GLA remains an ongoing debate.…”

Section: Introductionmentioning

confidence: 99%

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Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

McDaniel

Adams

Steele³

et al. 2023

Pediatric Blood & Cancer

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Kaposiform 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. KLA is a lymphatic anomaly and significant advancements have been made in understanding its pathogenesis and etiology since its first description in 2014. This review provides multidisciplinary, comprehensive, and state‐of‐the‐art information on KLA patient presentation, diagnostic imaging, pathology, organ involvement, genetics, and pathogenesis. Finally, we describe current therapeutic approaches, important areas for research, and challenges faced by patients and their families. Further insights into the pathogenesis of KLA may advance our understanding of other vascular anomalies given that similar signaling pathways may be involved.

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Section: Clinical Presentation and Imagingmentioning

confidence: 99%