Infants with Kasabach-Merritt syndrome do not have ''true'' hemangiomas
Infants with Kasabach-Merritt syndrome do not have ''true'' hemangiomas
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DOI：
10.1016/s0022-3476(97)70249-x
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发表时间：
1997-04-01
影响因子：
5.1
通讯作者：
Escande, JP
中科院分区：
文献类型：
--
作者：
Enjolras, O;Wassef, M;Escande, JP
目的：1940年，Kasabach和Merritt描述了一个患有血管异常、广泛性紫癜和血小板减少症的婴儿，他们称他的病变为“毛细血管瘤”。血管瘤是一种生长于婴儿期的良性肿瘤，其特征是内皮细胞增殖，在儿童期消退。尽管Kasabach-Merritt综合征（KMS）经常被认为是血管瘤的一种可能的并发症，但我们的经验表明，血小板减少症背后的解剖血管病变并不是婴儿和儿童期血管瘤的一种“真正的”、“经典的、退化的类型。回顾性分析22例KMS患者的临床资料、止血情况及治疗效果，并对其中15例活检标本进行分析。没有女性优势。所有患者都有严重的血小板减少症（最低血小板计数= 3000/mm（3））和纤维蛋白原消耗。在组织学上，没有一个婴儿具有典型的“毛细血管”，退化型血管瘤：它们表现为簇状血管瘤或卡波西状血管内皮瘤模式;所有标本还包含许多异常的类血管;淋巴管畸形是两名患者的主要组成部分。严重的发病率是存在的，我们的三名患者死亡，一个腿部截肢。“残留物”实际上是残留的血管瘤形成，持续时间可变，而不是典型的退化性血管瘤中稳定的纤维脂肪残留物;只有血液学现象在几年后才“治愈”，结论：KMS是一种独特的婴儿期疾病，但潜在的血管病变并不是一种典型的婴儿期血管瘤，这是一种不同的血管瘤，病理学上与簇状血管瘤或卡波西样血管内皮瘤相似，并伴有血管样血管。KMS的潜在病变是单一解剖实体还是异质性，不能从本研究中明确得出结论，我们需要更好地了解KMS的发病机制，以提高我们的治疗管理。
Objective: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion ''capillary hemangioma.'' Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood, Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a ''true,'' classic, involuting type of hemangioma of infancy and childhood.Study design: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them, Results: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm(3)) and consumption of fibrinogen. Histologically, none had the typical ''capillary,'' involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients, The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. ''Residua'' were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was ''cured'' after a period of years,Conclusions: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a ''true,'' classic, involuting type of hemangioma of infancy, This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels, Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study, We need a better understanding of the pathogenesis of KMS to improve our therapeutic management.