Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma
Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma
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DOI：
10.1097/00006534-199711000-00001
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发表时间：
1997-11-01
影响因子：
3.6
通讯作者：
Burrows, PE
中科院分区：
文献类型：
--
作者：
Sarkar, M;Mulliken, JB;Burrows, PE
患有大血管肿瘤和相关卡萨巴赫-梅里特凝血病的儿童对治疗的反应不一致，死亡率很高。为此，我们对21例此类患者进行了回顾性研究，重点关注临床、影像学和组织病理学特征。男女比例为1：1.6。 50% 的患者在出生时就发现有肿瘤；其余的则出现在整个婴儿期。位置为颈面部（n = 2）、肩部/上肢（n = 4）、躯干（包括腹膜后）（n = 11）和下肢（n = 4）。这些肿瘤迅速生长到很大，其特征是皮肤紫癜、水肿和边缘瘀斑进展。与普通血管瘤不同，磁共振成像显示弥漫性强化，边缘不明确，皮肤增厚，皮下脂肪绞合，含铁血黄素沉积，供引流血管较小。所有肿瘤均为卡波西样血管内皮瘤（KHE）；没有一例是婴儿血管瘤。光学显微镜显示不规则的小叶或形状不良的小血管通道渗透并捕获正常组织。典型特征包括纺锤形内皮细胞、周细胞和肥大细胞减少、微血栓和含铁血黄素沉积。电镜下可见宽的内皮细胞间隙和不完整的基底膜。 1 个肿瘤中扩张、增生、淋巴管通道明显。14 名婴儿的 KHE 接受了干扰素 α-2a 治疗：6 名加速消退；6 名婴儿加速消退。 2 增长趋于稳定； 6 人没有任何反应。死亡率为 24%（21 例中有 5 例）；这包括三名患有腹膜后KHE的婴儿。普通血管瘤不会发生Kasabach-Merritt现象。相反，它与更具侵袭性的 KHE 相关，很少与其他血管肿瘤相关。对当前药物治疗的不同反应凸显了我们对 KHE 血小板减少症发病机制的了解不足。
Children with a large vascular tumor and associated Kasabach-Merritt coagulopathy respond inconsistently to therapy and have a high mortality rate. For this reason, we undertook a retrospective study of 21 such patients, and focused on clinical, radiographic, and histopathologic features.The male to female ratio was 1:1.6. Tumor was noted at birth in 50 percent of patients; the remainder appeared throughout infancy. The location was cervicofacial (n = 2), shoulder/upper limb (n = 4), trunk including retroperitoneum (n = 11), and lower limb (n = 4). These tumors grew rapidly to large size and were characterized by cutaneous purpura, edema, and an advancing ecchymotic margin. In contrast to common hemangioma, magnetic resonance imaging showed diffuse enhancement with ill-defined margins, cutaneous thickening, stranding of subcutaneous fat, hemosiderin deposits, and small feeding and draining vessels.All tumors were Kaposiform hemangioendothelioma (KHE); none were infantile hemangioma. Light microscopy showed irregular lobules or sheets of poorly formed, small vascular channels infiltrating and entrapping normal tissues. Characteristic features included spindle-shaped endothelial cells, diminished pericytes and mast cells, microthrombi, and hemosiderin deposits. Wide endothelial intercellular gaps and incomplete basement membranes were seen by electron microscopy. Dilated, hyperplastic, lymphaticoid channels were prominent in one tumor.KHE in 14 infants was treated with interferon alpha-2a: 6 had accelerated regression; 2 had stabilization of growth; and 6 evidenced no response. The mortality rate was 24 percent (5 of 21); this included three infants with retroperitoneal KHE.Kasabach-Merritt phenomenon does not occur with common hemangioma. Rather it is associated with the more aggressive KHE and rarely with other vascular neoplasms. Variable response to current pharmacologic therapy underscores our inadequate knowledge of the pathogenesis of thrombocytopenia in KHE.