WC Chow, SY Ha, GCF Chan

Abstract

Vascular lesion with consumptive anaemia and thrombocytopenia (Kasabach Merritt phenomenon, KM phenomenon) is considered to be related to congenital vascular malformation rather than genuine haemangioma. Unlike the classical strawberry haemangioma, patients with KM phenomenon are often resistant to both steroid and interferon treatment, and the vascular malformation does not regress spontaneously. In the past 2 decades, various forms of treatment have been advocated but few with consistent effect. Recently vincristine, a commonly used chemotherapeutic agent with an anti-angiogenesis action, has demonstrated encouraging clinical effect in infants and young children with KM phenomenon with tolerable side effects. Recent data suggested that vincristine could control the vascular malformation in KM phenomenon if used early. However, its efficacy on patients with long-standing, refractory KM phenomenon remains unknown. We reported here a young adolescent girl with long and refractory KM syndrome who responded to weekly vincristine treatment. The thrombocytopenia resolved completely within 12 weeks of treatment and the size of the vascular malformation continued to shrink over a span of 1.5 years. Future study targeted at this group of patients will help to verify its efficacy in this clinical setting.

伴有消耗性凝血病和血小板減少的血管病變（Kasabach Merritt現象, KM現象）被認為與先天性血管畸形有關，而非真正的血管瘤。與經典的草莓性血管瘤不同，患有KM現象的病人對激素及干擾素治療無效，血管畸形不會自發轉歸。在過去的20年裏，提出過多種治療方式，但很少有確證的療效。目前，臨床顯示一種具有血管生成抑制作用的常用化療藥物，長春新鹼，對嬰幼兒KM現象有較好的療效而副作用可以耐受。最近的資料表明早期使用長春新鹼可以控制KM 現象中的血管畸形。但是，該藥對長期難治性KM現象的效果還不清楚。在此，我們報導1例患長期難治性KM綜合征的女性青少年對每週長春新鹼治療有反應。治療12周後血小板較少症完全好轉，1年半裏血管畸形持續縮小。以後對此類患者進行相關研究，將有助闡明長春新鹼對長期難治性KM現象的確切療效。