Clinical variability in multifocal lymphangioendotheliomatosis with thrombocytopenia: a review of the literature

Autor:	Judith F. Margolin, Ionela Iacobas, Kalyani R. Patel, Priya Mahajan, Joshua Manor
Rok vydání:	2021
Předmět:	Male Pathology medicine.medical_specialty Angiomatosis 03 medical and health sciences 0302 clinical medicine medicine Lymphatic vessel Humans Refractory Thrombocytopenia Multifocal lymphangioendotheliomatosis Lymphatic Vessels Sirolimus Lung business.industry Vascular malformation Infant Hematology medicine.disease Hyaluronan-mediated motility receptor Thrombocytopenia medicine.anatomical_structure Oncology 030220 oncology & carcinogenesis Pediatrics Perinatology and Child Health Choroid plexus Endothelium Lymphatic business Immunosuppressive Agents 030215 immunology medicine.drug
Zdroj:	Pediatric hematology and oncology. 38(4)
ISSN:	1521-0669
Popis:	Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a recently recognized disorder characterized by vascular lesions marked by distinct endothelial proliferation. Lesions affect multiple tissues, and MLT can be associated with refractory thrombocytopenia resulting in life-threatening bleeding. Diagnosing MLT may be challenging given its rarity and phenotypic variability. There is no consensus on the optimal management or treatment duration. We report a 4-month-old male who presented with multiple vascular malformations involving the gastrointestinal tract, lung, bones, choroid plexus, and spleen, with minimal cutaneous involvement and no thrombocytopenia. Wedge resection of a pulmonary nodule was strongly positive for lymphatic vessel endothelial hyaluronan receptor 1 favoring MLT despite the lack of thrombocytopenia. The patient's clinical symptoms and vascular lesions improved on sirolimus therapy. We review the literature to highlight the clinical variability of MLT and discuss the diagnostic and therapeutic options for MLT.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7ca80ac41cc3c4e5ef7ec2bda929c64e https://pubmed.ncbi.nlm.nih.gov/33641614 Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
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