Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly.
Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly.
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DOI：
10.1016/j.jpeds.2013.10.013
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发表时间：
2014-02
期刊：
影响因子：
--
通讯作者：
Trenor CC 3rd
中科院分区：
文献类型：
--
作者：
Croteau SE;Kozakewich HP;Perez-Atayde AR;Fishman SJ;Alomari AI;Chaudry G;Mulliken JB;Trenor CC 3rd
描述一种独特的组织学模式和预后不良的新淋巴疾病的临床和影像学特征。一项观察性、回顾性研究确定了1995年至2011年间在波士顿儿童医院血管异常中心就诊的20例具有明显淋巴组织病理学的患者。中位发病年龄为6.5岁（范围，出生-44岁）。临床和放射学表现提示一种全身性疾病。最常见的表现是呼吸道症状（50%）、止血异常（50%）和可触及的肿物（35%）。所有患者均有纵隔受累；19例患者出现心包积液（70%）和/或胸腔积液（85%）。胸外疾病见于骨和脾，较少见于腹部脏器、腹膜、被膜和四肢。尽管积极的程序和药物治疗，5年生存率为51%，总生存率为34%。从诊断到死亡的平均间隔时间为2.75年（范围为1至6.5年）。我们描述了一个临床病理上明显的淋巴异常。我们提出的术语卡样淋巴管瘤病（KLA），因为特征性簇状或片状纺梭状淋巴内皮细胞伴随畸形淋巴通道。胸内成分最常与发病率和死亡率有关；然而，胸外病变是常见的，表明KLA并不局限于肺淋巴。尽管积极的多模式治疗，KLA的死亡率仍然很高。
To describe the clinical and imaging characteristics of a new lymphatic disorder with a unique histological pattern and poor prognosis. An observational, retrospective study identified and characterized 20 patients with distinct lymphatic histopathology referred to the Vascular Anomalies Center at Boston Children’s Hospital between 1995 and 2011. The median age at onset was 6.5 years (range, birth-44 years). Clinical and radiologic findings suggested a generalized process. The most common presentations were respiratory symptoms (50%), hemostatic abnormalities (50%), and an enlarging, palpable mass (35%). All patients had mediastinal involvement; 19 patients developed pericardial (70%) and/or pleural effusions (85%). Extrathoracic disease manifested in bone and spleen and less frequently in abdominal viscera, peritoneum, integument, and extremities. Despite aggressive procedural and medical therapies, the 5-year survival was 51%, and the overall survival was 34%. Mean interval between diagnosis and death was 2.75 years (range, 1 to 6.5 years). We describe a clinicopathologically distinct lymphatic anomaly. We propose the term kaposiform lymphangiomatosis (KLA) because of characteristic clusters or sheets of spindled lymphatic endothelial cells accompanying malformed lymphatic channels. The intrathoracic component is most commonly implicated in morbidity and mortality; however, extra-thoracic disease is frequent, indicating that KLA is not restricted to pulmonary lymphatics. The mortality rate of KLA is high despite aggressive multi-modal therapy.
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影响因子：
5.6
作者：
ZUKERBERG, LR;NICKOLOFF, BJ;WEISS, SW
通讯作者：
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影响因子：
2.1
作者：
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影响因子：
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影响因子：
3.2
作者：
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通讯作者：
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影响因子：
2
作者：
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通讯作者：
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