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目的提高对卡-梅综合征(Kasabach-Merritt syndrome,KMS)的临床表现和治疗的认识。方法 2006年1月至2010年6月上海交通大学医学院附属新华医院收治KMS患儿10例,结合临床资料和文献,详细分析该病的病因、临床表现、诊断、治疗及预后。结果 KMS病因不明,病理生理基础是血小板减少和弥漫性血管内凝血(DIC),与巨大血管肿瘤密切相关;肿块的临床表现多样化;病理表现主要为Kaposiform血管内皮瘤和蔓状血管瘤;治疗方案包括纠正DIC和血小板减少,根据具体情况选择外科治疗(手术切除、栓塞等)或内科药物治疗(糖皮质激素、α-干扰素、免疫抑制剂等)去除血管肿瘤。结论婴儿血管瘤尤其是巨大血管瘤伴血小板减少时应警惕KMS的发生,一旦诊断明确,在对症治疗的基础上,根据不同血管瘤的大小、部位,采取不同的去除血管肿瘤治疗方法。     

儿童肿瘤常见急重症

随着肿瘤患儿的增多和化疗的进步,肿瘤及其治疗相关的急重症也逐渐为大家所熟悉.常见的有急性肿瘤溶解综合征(ATLS)、上腔静脉压迫综合征(SVCS)、粒细胞减少性脓毒症、弥漫性血管内凝血(DIC)、血栓性血小板减少性紫癜(TTP)、脑血管病变、急性脊髓压迫症等.     

Kasabach-Merritt现象

<正>Kasabach-Merritt现象(KMP)又称为巨大血管肿瘤-血小板减少综合征,是一组以巨大血管肿瘤伴发血小板显著减少、微血管溶血性贫血及消耗性凝血障碍为特征的综合征,由Kasabach和Merritt于1940年首先报道及命名。该病是血管肿瘤的一种罕见而严重的并发症,且多见于婴幼儿,在临床诊治过程中存在一定的困难,现将对该综合征的流行病学、发病机制、临床表现、诊断进行总结,并综述     

Kasabach-Merritt综合征治疗研究新进展

Kasabach-Merritt综合征(Kasabach-Merritt syndrome)由Kasabach和Merritt于1940年首次报道[1].目前,将巨大血管瘤合并血小板减少及全身紫癜等特点的症候群称为Kasabach-Merritt综合征,简称K-M综合征.本病多在新生儿期或小婴儿期发病,在婴幼儿血管瘤病人中发病率仅约0.3％[2,3].K-M综合征是一种威胁生命的消耗性凝血功能障碍疾病,死亡率在20％～30％,典型表现为巨大血管瘤伴血小板减少、低纤维蛋白血症等.主要并发症包括弥散性血管内凝血,气道压迫引起的呼吸衰竭,巨大肿瘤引起的高输出性心力衰竭等[4].K-M综合征一般可根据患儿血管瘤表现及肿物突然增大合并出血病史,并结合实验室检查确诊.而内脏血管瘤诊断较困难,故病人出现无法解释的血小板减少症和凝血功能障碍时应考虑K-M综合征可能.     

药物治疗卡-梅综合征

卡-梅综合征是一种巨大血管瘤伴有血小板减少和凝血因子消耗的综合征。血管瘤是造成血小板激活、血小板纤维蛋白形成的主要原因,同时伴有凝血因子消耗和纤维蛋白溶解。该病的治疗以控制凝血异常、血小板减少和根除血管瘤为主。具体方案包括全身使用糖皮质激素、放射线治疗、干扰素、免疫抑制剂及外科治疗(手术切除、栓塞、血管结扎等)。因病例较少且较分散,各种治疗效果及副作用报道不一。为此,该文简要综述国内外药物治疗卡-梅综合征的适应证、疗效及主要不良反应,旨在进一步寻找一个阶梯式的合理治疗方案,指导临床治疗,降低病死率。     

新生儿早发型败血症45例临床初步分析

目的 探讨新生儿早发型败血症的临床特征.方法 回顾分析2002年1月至2005年1月新生儿重症监护病房(NICU)收治早发型新生儿败血症45例,对其临床特征及非特异性实验室检查结果进行分析.结果 新生儿早发型败血症早期临床症状缺乏特异性,革兰阴性杆菌感染36例(80.0%),有26例(57.8%)并发多器官功能障碍综合征(MODS)或弥漫性血管内溶血(DIC),病死率26.7%;大肠埃希菌败血症患儿血白细胞＜5×109/L、血小板＜30×109/L、CRP≥60 μg/ml,并发多器官功能障碍综合征(MODS)、弥漫性血管内凝血(DIC)及死亡例数均高于肺炎克雷伯菌败血症患儿,并发MODS、DIC所用的时间短于肺炎克雷伯菌败血症,两组比较差异均有统计学意义,P值均＜0.05.结论 大肠埃希菌败血症比肺炎克雷伯菌败血症病情严重,易发生白细胞和血小板减少及CRP的增高,病死率高.因此对围产期有高危因素的新生儿生后应对感染性指标进行监测,早诊断早治疗.     

DIC前期诊治研究进展

弥散性血管内凝血 (DIC)是严重的出血综合征 ,依据常规实验室诊断标准确诊DIC时 ,病情已发展到中晚期 ,抢救极为困难。建立DIC前期概念在临床工作中非常重要。及早诊断 ,及时治疗 ,是提高DIC抢救成功率的关键。1　DIC前期 (Pre DIC)概念亦称前DIC ,DIC前状态。严重感染等因素可引起凝血因子和血小板处于动员与轻度消耗状态 ,并存在纤溶受抑 ,但尚未出现明显的出血症状 ,是常规化验检查异常的阶段 (DIC代偿期 ) ,易被忽视。它是初期凝血异常的短暂过程 ,恰恰又是治疗最有效的阶段 ,不治疗会很快发展为DIC[1] 。2　全国第六届血…     

儿童弥漫性血管内凝血研究进展

弥漫性血管内凝血(DIC)是由多种疾病和致病因素引起的临床综合征,以凝血因子和血小板被激活,大量促凝物质入血,凝血酶增加,进而微循环中形成广泛微血栓为特点.     

婴儿巨大血管瘤合并血小板减少综合征8例

目的总结8例婴儿巨大血管瘤合并血小板减少综合征（Kasabach—MerrittSyndrome，KMS）的治疗经验，探讨有效治疗婴儿巨大血管瘤合并血小板减少综合征的方法。方法回顾性分析本院收治的8例婴儿巨大血管瘤合并血小板减少综合征病例资料。血管瘤面积均占全身体表面积的10％（或面颈、关节等特殊部位相对巨大），血小板均在10×10^9／L以下。均应用激素、配合局部外科治疗或瘤体内药物注射，对与周围血管有交通的血管瘤，采用选择性经皮大块缝扎法，待血小板回升后配合局部注射治疗。结果4例治愈，2例好转，2例无效（其中1例死亡，另1例失访）。结论巨大KMS目前尚无标准治疗方法，本组采用三级治疗方案早期进行综合治疗有一定临床疗效。     

新生儿弥散性血管内凝血的诊治进展

弥散性血管内凝血(disseminated intravascular coagulation,DIC)是指发生于许多疾病过程中的一种获得性出血综合征.新生儿DIC绝大多数为急性,且较严重.早期诊断、及时治疗是提高新生儿DIC治愈率的关键.近年来随着对DIC研究的深入,新生儿DIC的诊断及治疗均有了较大进展.     

Giant cavernous haemangioma with Kasabach-Merritt syndrome: a case report and review

We report the case of a 4-month-old boy presenting with a giant cutaneous haemangioma complicated by Kasabach-Merritt syndrome (KMS) with severe thrombocytopenia. After poor response to corticosteroid therapy and subsequent treatment with interferon alpha-2a, radiotherapy led to tumour regression and resolution of the disseminated intravascular coagulopathy over a 14-month period of follow up. Whereas the various available treatment options are reviewed and discussed in this article, the therapy of choice should be chosen individually. CONCLUSION: to date prospective randomised and controlled trials are required to investigate the optimal management of patients with Kasabach-Merritt syndrome.     

Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia

The authors report Kasabach-Merritt syndrome (KMS) in a patient with thrombocytopenia and splenic hemangioma. A 13-month-old boy with a history of anemia, thrombocytopenia, and abdominal mass was admitted to the hospital. The scintigraphic studies showed that a large mass contiguous to the spleen was responsible for the platelet uptake. After partial splenectomy, the platelet count returned to normal. This report of KMS in a child with splenic hemangioma suggests that the scintigraphic studies are mandatory to confirm diagnosis. Indium-111-labeled platelets are useful in identifying hemangiomatous sequestration of platelets in patients with thrombocytopenia.     

血管瘤伴血小板减少综合征11例

目的总结Kasabach-Merritt综合征的的临床特点,提高对本病的认识并探讨其治疗方案。方法对2002·4-2006·3年我院收治的11例Kasabach-Merritt综合征病人的临床特点、实验室检查、治疗情况进行回顾性分析。结果11例Kasabach-Merritt综合征中,男4例,女7例,年龄8天~5岁,临床特点为不同部位、大小的血管瘤伴不同程度血小板减少及出血症状,部分伴发骨骼畸形。治疗结果为1例手术治愈,8例药物治疗临床症状改善,2例药物治疗死亡。结论Kasabach-Merritt综合征临床少见,误诊率高,出血重,病死率相对较高。依据血小板减少程度不同,采取不同治疗方案,缓解病情,以提供适时的手术时机、对本症的预后尤为重要。     

Successful low‐dose radiotherapy treatment for Kasabach–Merritt syndrome

Kasabach–Merritt syndrome (KMS) is characterized by hemangioma associated with life‐threatening thrombocytopenia, and is a consumptive coagulopathy. Although treatments available include corticosteroids, α‐interferon, vincristine, and surgery, response may be unsatisfactory, and the mortality rate remains at approximately 30%. Although radiotherapy has been used effectively for KMS, it may cause growth retardation and secondary malignancy. We report a case of KMS in which hemangioma of the left thigh was successfully treated with low‐dose radiotherapy (6 Gy in six fractions, weekly) after failure of corticosteroid therapy. No significant late effects due to the radiotherapy were noted at 5 year follow up. Thus, low‐dose radiotherapy remains an important treatment method for KMS when patients fail to respond to other treatments.     

Chronic consumption coagulopathy due to an occult splenic haemangioma: Kasabach-Merritt syndrome

We report an 11-year-old girl with a 2-year histor of bruising associated with thrombocytopenia and dysfibrinogenaemia. On admission she presented with a large subcutaneous haematoma and splenomegaly and was severely anaemic. Laboratory investigations revealed signs of consumption coagulopathy. Radiological examination showed splenic, retroperitoneal and intra-ossal haemangiomas. After splenectomy, platelet count and coagulation parameters returned to normal.Conclusion Contrary to widely held views, occult visceral haemangioma can lead to Kasabach-Merritt syndrome beyond infancy and is not necessarily associated with visible cutaneous haemangioma. It should be included in the differential diagnosis of chronic thrombocytopenia at any age. Early determination of fibrinogen degradation product levels is advised in order to detect an underlying chronic consumption coagulopathy prompted by an extensive search for multifocal liaemangioma.     

GIANT HAEMANGIOMA WITH A DISORDER OF COAGULATION

The mechanism of the coagulation disorder in an infant with a large haemangioma of the right arm has been studied. The disorder was characterised by thrombocytopenia, prolonged bleeding time and the occurrence of fibrinolytic split products in the blood. The levels of various coagulation factors were normal. The mechanism of the disorder was further investigated by injection of ⁵¹Cr labelled platelets, which disappeared very rapidly from the circulation owing to the uptake by the haemangioma. Injection of ¹²⁵I labelled fibrinogen revealed a considerable increase in disappearance rate, which, however, was not as high as for ⁵¹Cr labelled platelets. Treatment with heparin in this case appeared inadvisable because the thrombocytopenia was found to be due mainly to mechanical destruction in the haemangioma while intravascular coagulation played only a subordinate role. On the other hand, the fibrinolytic activity in the tumour vessels was found to be high, which together with the occurrence of fibrinolytic split products in the blood was considered to indicate treatment with inhibitors of fibrinolysis (EACA). During this treatment the angioma decreased in size. It would therefore appear advisable to investigate the mechanisms of the coagulation disorders in these patients before deciding upon treatment.     

Massive hepatic congenital haemangioma: clinical dilemmas

A case of massive hepatomegaly secondary to a hepatic congenital haemangioma in a preterm neonate is described. This infant died after withdrawal of neonatal intensive care support, following massive intracerebral haemorrhage. There remains considerable uncertainty regarding the classification of, and therapy for, hepatic vascular anomalies.     

Haemangioma with coagulopathy: sustained response to prednisone.

Two cases of giant haemangioma with thrombocytopenia are described. A satisfactory and sustained response to prednisone was achieved in respect of both coagulation abnormalities and tumour size. It is proposed that prednisone is effective by enhancement of thrombosis and reduction of fibrinolysis within the tumour, thus promoting a natural form of resolution. It is suggested that prednisone therapy should be first-choice treatment of complicated haemangiomas.     

Hydrops foetalis caused by hepatic haemangioma

The authors report a moderately premature baby with Down's syndrome and hydrops, the latter probably caused by a large hepatic haemangioma which was diagnosed only after birth. At birth the baby was affected by massive right hydrothorax, ascites, hypoalbuminaemia and severe respiratory distress. With the use of modern neonatal intensive care, the baby survived. Corticosteroid treatment (prednisolone 2 mg kg⁻¹ d⁻¹ i.v. in divided doses) was associated with a very rapid resolution of the haemangioma and the baby was healthy at follow-up. Although hepatic angiomas are not uncommon in the neonatal period, the association with hydrops is a rare finding.