{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T16:03:03Z","timestamp":1776960183278,"version":"3.51.4"},"reference-count":8,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2014,1,1]],"date-time":"2014-01-01T00:00:00Z","timestamp":1388534400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Disorders of the pulmonary lymphatic system include macro- and microcystic lymphatic malformations, primary or secondary lymphangiectasias, generalized lymphatic anomalies, diffuse pulmonary lymphangiomatosis, and combinations of lymphatic and other tissue anomalies, including lymphangioleiomyomatosis (LAM). We report a case of a patient with a newly defined entity classified as kaposiform lymphangiomatosis (KLA). This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. Laboratory evaluation demonstrated a low normal platelet count, elevated d-Dimer, low normal fibrinogen, and elevated fibrin split products. Chest computerized tomography imaging showed enlarged hypodense lymph nodes in the mediastinum and hila, and peribronchovascular thickening, without evidence of cystic parenchymal lesions. Magnetic resonance imaging of the chest showed cystic mediastinal lymph nodes with heterogeneously increased T2 and decreased T1 signal intensity. Fiberoptic bronchoscopy revealed hyperemic mucosa with granular appearance suggestive of a submucosal infiltrative process. Pathological specimens revealed dilated, malformed lymphatic channels within the pleura, pulmonary septa, and bronchovascular bundles, and foci of perilymphatic and intralymphatic spindle cells which reacted with the Prospero homeobox protein 1 (PROX-1) immunostain. The morphology and immunohistochemistry results were consistent with a diagnosis of KLA. This newly recognized clinical-pathological entity among intrathoracic lymphatic anomalies is distinguished from generalized lymphatic anomaly and diffuse pulmonary lymphangiomatosis in part by characteristic hematological abnormalities and hemorrhagic complications, including hemoptysis, as experienced by our patient.<\/jats:p>","DOI":"10.1513\/annalsats.201308-287bc","type":"journal-article","created":{"date-parts":[[2014,2,14]],"date-time":"2014-02-14T10:04:54Z","timestamp":1392372294000},"page":"92-95","source":"Crossref","is-referenced-by-count":35,"title":["Kaposiform Lymphangiomatosis, a Newly Characterized Vascular Anomaly Presenting with Hemoptysis in an Adult Woman"],"prefix":"10.1093","volume":"11","author":[{"given":"Fadi","family":"Safi","sequence":"first","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anita","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Pathology and Lab Medicine and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Denise","family":"Adams","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vasuki","family":"Anandan","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Toledo Medical Center, Toledo, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francis X.","family":"McCormack","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati School of Medicine, Cincinnati, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ragheb","family":"Assaly","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2014,1,1]]},"reference":[{"key":"2026012303553811100_bib1","doi-asserted-by":"crossref","first-page":"1313","DOI":"10.1016\/0046-8177(93)90265-I","article-title":"Diffuse pulmonary lymphangiomatosis","volume":"24","author":"Tazelaar","year":"1993","journal-title":"Hum Pathol"},{"key":"2026012303553811100_bib2","article-title":"Kaposiform lymphangiomatosis: clinical features of 23 patients with a distinct aggressive lymphatic disorder [abstract]","author":"Croteau","year":"2013"},{"key":"2026012303553811100_bib3","article-title":"Kaposiform lymangiomatosis: a new entity distinct from generalized lymphatic anomaly, kaposiform hemangioendothelioma, lymphedema-associated angiosaroma, and lymphangioma-like kaposi sarcoma","author":"Perez-Atayde","year":"2012"},{"key":"2026012303553811100_bib4","first-page":"269","article-title":"Kaposiform lymphangiomatosis (KLA): an aggressive variant of lymphangiomatosis [abstract]","volume":"84","author":"Debelenko","year":"2004","journal-title":"Lab Invest"},{"key":"2026012303553811100_bib5","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1007\/s00247-011-2124-1","article-title":"Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign","volume":"41","author":"Shah","year":"2011","journal-title":"Pediatr Radiol"},{"key":"2026012303553811100_bib6","doi-asserted-by":"crossref","first-page":"1548","DOI":"10.1097\/PAS.0b013e31825eae67","article-title":"Diffuse pulmonary lymphatic disease presenting as interstitial lung disease in adulthood: report of 3 cases","volume":"36","author":"Boland","year":"2012","journal-title":"Am J Surg Pathol"},{"key":"2026012303553811100_bib7","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00000478-200405000-00001","article-title":"Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma","volume":"28","author":"Lyons","year":"2004","journal-title":"Am J Surg Pathol"},{"key":"2026012303553811100_bib8","author":"Safety and efficacy study of sirolimus in complicated vascular anomalies"}],"container-title":["Annals of the American Thoracic Society"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/annalsats\/article-pdf\/11\/1\/92\/66485452\/annalsats_11_1_92.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/annalsats\/article-pdf\/11\/1\/92\/66485452\/annalsats_11_1_92.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T08:55:54Z","timestamp":1769158554000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/annalsats\/article\/11\/1\/92\/8432038"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,1,1]]},"references-count":8,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2014,1,1]]},"published-print":{"date-parts":[[2014,1,1]]}},"URL":"https:\/\/doi.org\/10.1513\/annalsats.201308-287bc","relation":{},"ISSN":["2329-6933","2325-6621"],"issn-type":[{"value":"2329-6933","type":"print"},{"value":"2325-6621","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2014,1]]},"published":{"date-parts":[[2014,1,1]]}}}