Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels.
Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels.
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DOI：
10.1002/pbc.28529
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发表时间：
2020-09
影响因子：
3.2
通讯作者：
Le Cras TD
中科院分区：
文献类型：
--
作者：
Crane J;Manfredo J;Boscolo E;Coyan M;Takemoto C;Itkin M;Adams DM;Le Cras TD
Kaposiform淋巴管瘤病（KLA）是一种罕见的，危及生命的先天性淋巴管畸形。诊断往往被延误，由于复杂的模糊症状。血液血管生成素-2（ANG2）水平在KLA中升高，可用作监测疾病状态的生物标志物。我们报告一个7岁的男孩容易瘀伤，腹股沟肿胀，和消耗性凝血功能障碍，诊断为KLA。采用泼尼松、西罗莫司、长春新碱和唑来膦酸盐联合治疗。血浆ANG2水平在诊断时高度升高，但在治疗期间降低。该患者在38个月期间显示出显著的临床改善，ANG2水平的正常化与凝血病的消退相关。
Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. We report a 7-year-old male child with easy bruising, inguinal swelling, and consumptive coagulopathy, diagnosed with KLA. A multimodal treatment regimen of prednisone, sirolimus, vincristine, and adjunctive zoledronate was used. Plasma ANG2 levels were highly elevated at diagnosis but decreased during treatment. The patient showed significant clinical improvement over a 38-month period and normalization of ANG2 levels correlated with resolution of the coagulopathy.
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影响因子：
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作者：
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通讯作者：
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影响因子：
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