Division of Pediatric Hematology/Oncology, Boston Children's Hospital, Boston, MA 02115, USA.
J Pediatr. 2013 Jan;162(1):142-7. doi: 10.1016/j.jpeds.2012.06.044. Epub 2012 Aug 4.
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).
A 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 ∼0.91 case per 100000 children. KHE manifested in infancy in 93% of cases, with 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 cutaneous 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.
研究卡波西样血管内皮细胞瘤（KHE）患者的临床表现，以描述疾病谱和卡-梅现象（KMP）的危险因素。
对 1991 年至 2009 年间在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，确定了 107 例具有足够纳入数据的患者。
马萨诸塞州 KHE 的患病率约为每 10 万名儿童中 0.91 例。93%的 KHE 病例在婴儿期发病，60%为新生儿。常见的表现特征包括皮损增大（75%）、血小板减少（56%）和肌肉骨骼疼痛或功能减退（23%）。皮肤 KHE 好发于四肢，尤其是关节上方。在我们的队列中，71%的患者发生 KMP（11%在初次就诊后发生），11%的患者缺乏皮肤表现。虽然腹膜后和胸腔内病变较少见，但分别有 85%和 100%的病例因 KMP 而复杂化。与浅表病变相比，浸润肌肉或更深部的 KHE 发生 KMP 的可能性增加 6.3 倍，如果是腹膜后或胸腔内病变则增加 18 倍。局限于骨骼或在婴儿期后出现的 KHE 不会发生 KMP。
婴儿期 KHE 最常见的临床表现是增大的皮肤病变。年龄较大的 KHE 患者或无皮肤表现的患者会出现肌肉骨骼症状或不典型症状。当肿瘤浸润肌肉或发生在腹膜后或纵隔时，KMP 的风险会显著增加。
