Kaposiform Lymphangiomatosis: Unifying Features of a Heterogeneous Disorder
Kaposiform Lymphangiomatosis: Unifying Features of a Heterogeneous Disorder
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DOI：
10.1002/pbc.25278
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发表时间：
2015-05-01
影响因子：
3.2
通讯作者：
Meier, Emily Riehm
中科院分区：
文献类型：
--
作者：
Fernandes, Violet M.;Fargo, John H.;Meier, Emily Riehm
卡波西样淋巴管瘤病 (KLA) 是一种罕见的异常淋巴管增生，常并发胸膜/心包积液和消耗性凝血病，可能导致危及生命的出血。由于临床异质性以及实验室值、放射学特征和病理特征的可变结果，建立诊断具有挑战性。我们报告了三名患者，他们的症状缓慢进展，并出现胸腔或心包积液，这是消耗性凝血病和贫血的证据。尽管 KLA 是一种罕见且具有挑战性的诊断，但对于出现非特异性惰性症状、胸腔或心包积液以及消耗性凝血病实验室证据的患者，应考虑 KLA。儿科血癌 2015；62：901-904。 (c) 2014 年 Wiley 期刊公司。
Kaposiform 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 features, and pathologic characteristics. We report three patients who had slowly progressive symptoms and presented with pleural or pericardial effusions, evidence of a consumptive coagulopathy and anemia. Despite being a rare and challenging diagnosis, KLA should be considered in patients presenting with non-specific indolent symptoms, pleural or pericardial effusions and laboratory evidence of a consumptive coagulopathy. Pediatr Blood Cancer 2015;62:901-904. (c) 2014 Wiley Periodicals, Inc.