Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.

Autor: McDaniel CG; University of Cincinnati College of Medicine, Cincinnati, Ohio, USA., Adams DM; Division of Oncology, Comprehensive Vascular Anomalies Program, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA., Steele KE; Collaborative Research Advocacy for Vascular Anomalies Network (CaRAVAN), Bethesda, Maryland, USA., Hammill AM; Division of Hematology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA., Merrow AC; Department of Radiology and Medical Imaging, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.; Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA., Crane JL; Division of Pediatric Endocrinology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Smith CL; Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA., Kozakewich HPW; Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA., Le Cras TD; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.; Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2023 Apr; Vol. 70 (4), pp. e30219. Date of Electronic Publication: 2023 Jan 22.
DOI: 10.1002/pbc.30219
Abstrakt: 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.
(© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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