Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals.
Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals.
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DOI：
10.1016/j.jpeds.2012.06.044
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发表时间：
2013-01
影响因子：
5.1
通讯作者：
Trenor, Cameron C., III
中科院分区：
文献类型：
--
作者：
Croteau, Stacy E.;Liang, Marilyn G.;Kozakewich, Harry P.;Alomari, Ahmad I.;Fishman, Steven J.;Mulliken, John B.;Trenor, Cameron C., III
目的探讨卡泊西样血管内皮瘤（KHE）患者的表现特征，以描述卡萨巴赫-梅里特现象（KMP）的疾病谱和危险因素。对1991年至2009年期间到波士顿儿童医院血管异常中心就诊的163例KHE患者进行回顾性分析，确定107例患者有足够的资料纳入。马萨诸塞州KHE患病率约为0.91/10万名儿童。93%的KHE表现在婴儿期；60%是新生儿。常见的表现包括皮肤病变扩大（75%），血小板减少（56%），肌肉骨骼疼痛或功能下降（23%）。皮肤KHE多发于四肢，尤其是关节上。在我们的队列中，71%的患者发展为KMP（11%在初次就诊后），11%的患者没有皮肤发现。腹膜后和胸内病变虽然不太常见，但分别有85%和100%的病例并发KMP。与浅表病变相比，KHE浸润到肌肉或更深部位发生KMP的可能性高6.3倍，腹膜后或胸内的可能性高18倍。局限于骨骼或婴儿期后出现的KHE未表现为KMP。病变扩大是婴儿期KHE最常见的表现特征。老年KHE患者或无皮肤表现的患者表现为肌肉骨骼主诉或非典型症状。当肿瘤浸润肌肉或KHE出现在腹膜后或纵隔时，KMP的风险显著增加。
To examine the presentation characteristics of patients with kaposiform hemangioendothelioma (KHE) to describe the spectrum of disease and risk factors for Kasabach-Merritt phenomenon (KMP). Retrospective review of 163 patients referred to the Vascular Anomalies Center at Children’s Hospital Boston for KHE between 1991 and 2009 identified 107 patients with sufficient data for inclusion. The prevalence of KHE in Massachusetts is approximately 0.91/100,000 children. KHE manifested in infancy in 93% of cases; 60% as neonates. Common presenting features included enlarging cutaneous lesion (75%), thrombocytopenia (56%), and musculoskeletal pain or decreased function (23%). Cutaneous KHE favored the extremities, especially overlying joints. In our cohort 71% developed KMP (11% after initial presentation), and 11% of patients lacked cutaneous findings. Retroperitoneal and intrathoracic lesions, though less common, were complicated by KMP in 85% and 100% of cases, respectively. Compared with superficial lesions, KHE infiltrating into muscle or deeper was 6.3 fold more likely to manifest KMP, and 18-fold higher if retroperitoneal or intrathoracic. KHE limited to bone or presenting after infancy did not manifest KMP. An enlarging lesion is the most common presenting feature of KHE in infancy. Older patients with KHE or those lacking cutaneous manifestations present with musculoskeletal complaints or atypical symptoms. The risk of KMP increases dramatically when tumor infiltrates muscle or when KHE arises in the retroperitoneum or mediastinum.
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影响因子：
--
作者：
Dompmartin A;Acher A;Thibon P;Tourbach S;Hermans C;Deneys V;Pocock B;Lequerrec A;Labbé D;Barrellier MT;Vanwijck R;Vikkula M;Boon LM
通讯作者：
Boon LM
影响因子：
5.6
作者：
ZUKERBERG, LR;NICKOLOFF, BJ;WEISS, SW
通讯作者：
WEISS, SW
DOI：
10.1001/archpedi.1940.01990160135009
发表时间：
1940-05-01
影响因子：
--
作者：
Kasabach, HH;Merritt, KK
通讯作者：
Merritt, KK
DOI：
10.1002/hed.20414
发表时间：
2006-08-01
影响因子：
2.9
作者：
Birchler, Manfred T.;Schmid, Stephan;Gysin, Claudine
通讯作者：
Gysin, Claudine
影响因子：
3.6
作者：
Sarkar, M;Mulliken, JB;Burrows, PE
通讯作者：
Burrows, PE