卡波西样血管内皮瘤伴卡梅综合征1例报告与文献复习

目的 了解卡波西样血管内皮瘤（Kaposiformhemangio-endothelioma,KHE）伴卡梅综合征（Kasabach-Merritt syndrome,KMS）的诊断、临床特点及治疗方法.方法 分析采用甲强龙＋长春新碱（VCR）＋环磷酰胺（CTX）治疗1例2012年2月我院收治的经病理诊断KHE伴KMS的临床疗效,并复习相关文献.结果 （1）化疗方案采用甲强龙＋ VCR＋ CTX.2周血小板恢复正常.观察至2012年6月患儿血小板及凝血功能恢复正常,左侧肩关节活动改善,复查肩部CT提示肿瘤明显缩小.（2）化疗后患儿出现双上眼睑下垂,经营养神经治疗后恢复正常.长春新碱毒副作用可逆.结论 KHE是一种临床罕见,但以儿童和婴幼儿为主要发病年龄的低度恶性血管源性肿瘤,临床上常伴有KMS.本病例提示对于难以手术治疗的患儿可给予激素及免疫抑制剂的联合治疗,能改善KMS并肿瘤缩小.     

Kasabach-Merritt综合征5例临床分析

目的：分析5例婴儿Kasabach-Merritt综合征（KMS）临床数据及转归。方法1993年6月-2012年2月期间我科收治的5例KMS患儿（男4例,女1例）,回顾性收集上述病例的一般资料、血常规及出凝血检查、影像学和病理学检查结果并加以分析。结果5例患儿中3例为皮下型巨大血管瘤,2例为内脏型巨大血管瘤,除1例首诊时确诊为KMS外,其余4例首诊时均存在误诊。血液检查结果提示所有患儿均存在不同程度消耗性凝血功能障碍。所有患者均给予肾上腺糖皮质激素治疗及对症支持治疗,对激素敏感者3例,激素敏感率为60％,手术治疗1例。经治疗存活4例,死亡1例,死亡原因为严重DIC伴多脏器功能衰竭。结论应提高对KMS的认识以减少误诊发生率,对婴儿难治性血小板减少伴凝血功能异常者需注意除外KMS ,不同患儿对药物的治疗反应存在个体化差异,故应针对不同情况采用个体化综合治疗方法,以提高临床诊疗效果。     

婴儿血管瘤并发卡-曼综合征的血管栓塞术治疗

目的探讨婴儿血管瘤合并消耗性凝血病(卡-曼综合征)血管栓塞术的临床应用。方法以选择性栓塞方法,对4例血管瘤合并卡-曼综合征的患儿实施血管瘤的病理血管床和供血动脉的栓塞。结果3例患儿接受栓塞治疗后1～2周内血管瘤明显缩小,局部软组织充血、肿胀迅速缓解并局限,血小板快速回升并稳定在正常水平,卡-曼综合征获得有效控制。1例于栓塞术后2周血小板再次快速回落,经补充栓塞治疗后病情得到缓解。结论血管栓塞术对血管瘤合并消耗性凝血病有着较为理想的疗效,对于外科手术治疗困难合并有卡-曼综合征的血管瘤患儿,倡导给予及时的血管栓塞术治疗。     

婴儿突发皮肤紫癜

患儿,男,2个月,因发现皮肤紫癜、血小板减少2d就诊,体查发现背部紫红色肿块,软组织彩超提示组织内丰富血流信号,骨髓细胞学提示巨核细胞增多,经激素及丙种球蛋白治疗后血小板迅速上升并维持在正常;同时口服普萘洛尔,随访4个月,患儿背部瘤体明显减小。血管瘤和免疫性血小板减少症诊断明确。对于血管瘤伴血小板减少的患者,应增强对血管瘤血小板减少症 （KMS）的认识,拓展思路,理顺血小板减少与血管瘤的关系,警惕其它原因所致的血小板减少。     

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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。     

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。     

尿素与甲基强的松龙联合介入治疗颌面部重症血管瘤伴血小板减少

目的 探讨婴幼儿颌面部重症血管瘤伴血小板减少的治疗方法 .方法 收集分析2001至2006年我科收治的 35 例颌面部重症血管瘤伴血小板减少患儿的临床表现及治疗经过,年龄最小10 d,最大5个月,平均31 d,血小板明显减低,在76×109/L以下,最低至6.2×109/L,采用颈外动脉结扎置管、40%尿素溶液与甲基强的松龙针剂联合介入治疗的方法 .平均随访1年8个月.结果 35 例患儿中,血小板计数3～7 d恢复到正常的 30 例,8～11 d 5例;血管瘤瘤体逐渐缩小,6～12个月消失 28 例,13～23个月 7 例.其中 3 例出现病情反复.结论 颈外动脉结扎置管、尿素与甲基强的松龙联合介入治疗婴幼儿颌面部重症血管瘤伴血小板减少,疗效确切,创伤小,副作用少.本疗法操作简便,不需要特殊设备,易于推广,是一种较为理想的治疗小儿颌面部重症血管瘤伴血小板减少的方法 .     

儿童部分脾栓塞长期疗效观察

目的：总结部分脾栓塞治疗儿童脾功能亢进的长期效果。方法：１７例功能亢进患儿施行了部分脾栓塞，全部病例随访５－６年。结果：栓塞后第３天内，患儿白细胞及血小板计数显著升高，在随访的６年期间血小板及白细胞计数的仍维持在正常范围，且脾脏亦显著地缩小，结论：部分脾栓塞是替代脾切除治疗儿童脾功能高进的有效方法。     

婴幼儿丛状血管瘤24例

目的 探讨婴幼儿丛状血管瘤(TA)的临床特点、疾病进程,及并发Kasabach-Merritt现象(KMP)的治疗方法.方法 收集、分析2009年1月至2013年3月收治的24例婴幼儿TA患儿的临床资料和随访资料.男10例,女14例;就诊年龄18 d～2岁,中位年龄为7.5个月.根据病变情况选择临床观察及手术治疗等.随访1.2 ～5.4年,平均3.6年.随访观察患儿病情变化.结果 临床常见症状或体征包括暗红或紫红色边界不清的体表皮损、血小板减少、疼痛或功能受限以及多汗、多毛等.其临床进程主要有3种类型:病变完全或部分自发性消退2例(8.3％)、持续存在9例(37.5％)、合并KMP 13例(54.2％).TA发病至并发KMP平均间隔时间45.2 d(0d～4个月).KMP发病最初症状或体征表现为迅速增大的瘤体(8例)、瘤体明显变厚张力增大(3例)、呼吸道窘迫表现为呼吸急促、三凹征明显(2例).13例合并KMP均采用手术治疗,手术完全切除者10例,术后血小板计数1～3d升至正常,血红蛋白及凝血功能1～2周逐渐恢复正常;大部切除者3例,血小板计数术后均出现反复,但明显高于术前,多维持在60×109/L以上,经术后给予药物治疗,其中2例3～6个月后瘤体逐渐消失,血小板计数恢复正常,1例术后因多器官功能衰竭死亡.结论 积极主动干预治疗主要适用于影响外观或容貌、已经或可能并发KMP或其他功能异常.对年龄较小无严重并发症的TA,早期可以随访观察,定期监测其血小板计数,以尽早发现KMP.对合并KMP的TA,明确诊断后尽早手术治疗,可明显缩短治疗时间,降低药物不良反应,治疗效果良好,降低患儿病死率.     

普萘洛尔治疗婴幼儿呼吸道血管瘤的疗效分析

目的 分析口服普萘洛尔治疗婴幼儿呼吸道血管瘤的效果。方法 回顾性分析2012年11月至2019年12月收治的、经支气管镜及喉部平扫增强CT/MRI确诊、且口服普萘洛尔治疗的婴幼儿呼吸道血管瘤患儿的临床资料。结果 共纳入20例患儿,所有患儿口服普萘洛尔治疗1~2 d后喉喘鸣、呼吸困难等症状均有改善,中位治疗时间为10个月（范围：6~12个月）。中位随访时间为10个月（范围：3~15个月）。19例（95%）患儿瘤体基本消退;1例（5%）患儿停药6个月后复查瘤体较停药前增大,予增加普萘洛尔剂量治疗6个月后,病情未出现反复;仅1例（5%）患儿出现不良反应;1例（5%）患儿尚在治疗中。结论 口服普萘洛尔可快速缓解呼吸困难等症状,使瘤体消退,不良反应少,可有效治疗婴幼儿呼吸道血管瘤。     

Successful treatment by coil embolization for infantile hemangioma with Kasabach–Meritt syndrome of newborn

Infantile hemangioma (IH) is the most common tumor of infancy, and it sometimes associated with Kasabach–Meritt syndrome (KMS) characterized by anemia, intraperitoneal hemorrhage secondary to rupture, coagulopathy, jaundice, and vascular malformations involving the brain, skin, gut, and other organs. Here, we report two newborn patients having IH with KMS at birth. The first patient had a giant hemangioma in the liver, which was successfully treated with i.v. corticosteroid and coil embolization. The second patient had a large hemangioma of the right axillary region, which was also successfully treated with i.v. corticosteroid, beta‐blocker, coil embolization and local irradiation. All symptoms were controlled without any side‐effects in both patients. According to these findings, combination therapy including coil embolization and corticosteroid is effective for IH patients with KMS. The indications for and timing of coil embolization should be determined further cases have been accumulated.     

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.     

儿童Kasabuch—Merritt综合征手术治疗探讨

目的评价儿童Kasabuch—Merritt综合征（Kasabuch—Merritt svndrome,K—M综合征）的手术治疗效果,探讨K—M综合征患儿的治疗方法。方法回顾性分析本院2001年7月至2008年7月经手术治疗的14例K—M综合征患儿的临床资料。患儿血小板最低值为3～29×10^9／L。均先采取保守治疗,疗效欠佳,而转手术切除瘤体。结果瘤体完整切除8例,部分切除6例。手术后1周,9例血小板计数〉100×10^9／L,3例为70～100×10^9／L,1例为58×10^9／L。1例术后因多器官功能衰竭死亡。对13例存活患儿随访6个月至5年,患儿血小板均恢复并保持正常。5例肿块部分残留者肿块逐渐消退。结论对瘤体巨大、药物治疗难以维持血小板正常的K—M综合征患儿,如病变部位及全身状况允许,应尽早手术治疗,完整或部分切除瘤体,可达到理想的治疗效果。     

Treatment of the Kasabach-Merritt syndrome with pegylated recombinant human megakaryocyte growth and development factor in mice: elevated platelet counts, prolonged survival, and tumor growth inhibition.

Kasabach-Merritt Syndrome (KMS) is seen in children with large vascular tumors. KMS is characterized by very low platelet counts and a consumption of coagulation factors causing life-threatening complications. It has been proposed that thrombopenia in these patients is caused by intratumoral trapping of platelets. The truncated form of the cMpl-receptor ligand thrombopoietin, pegylated human megakaryocyte growth and development factor (Peg-rHuMGDF), is an agent that stimulates platelet production. We hypothesized that stimulation of the platelet production would prevent the life-threatening complications of patients with KMS owing to low platelet counts. In a mouse model of KMS, with tumors derived from a hemangioendothelioma cell line, we studied the effect of Peg-rHuMGDF. Treatment with Peg-rHuMGDF (10 microg/kg/day intraperitoneally) increased platelet counts by 7-8-fold compared with control tumor-bearing mice after 11 d of treatment (p < 0.001, n = 8). Survival was significantly increased, with 50% of treated animals alive at 1 mo versus 0% in untreated controls. Interestingly, we also observed an inhibition of tumor growth by 75% (p < 0.001, n = 8). Hematoxylin and eosin staining showed fresh fibrin clots in the treated tumors, suggesting that higher platelet counts caused intravascular thrombosis of tumor vessels. We conclude that increased platelet production in this model of KMS resulted in an antivascular tumor effect via platelet trapping. Further, we propose that thrombopoietin may be of critical value in preventing life-threatening complications from KMS.     

Endovascular treatment of huge cervicofacial hemangioma complicated by Kasabach-Merritt syndrome

A 2-month-old girl with a huge cervicofacial hemangioma complicated by Kasabach-Merritt syndrome was presented. Two weeks corticosteroid treatment was ineffective, and deterioration of coagulation parameters and compromise of the airway and left vision due to huge hemangioma prompted use of endovascular treatment. Transfemoral arterial embolization using polyvinyl alcohol particles resulted in rapid clinical improvement. Endovascular treatment should be considered as an important therapeutic option for hemangioma complicated by Kasabach-Merritt syndrome.     

Hemangioma thrombocytopenia syndrome. A case masquerading as an encephalocele

The association of hemangioma and thrombocytopenia has been recognized since 1940. We cared for a newborn boy whose hemangioma resembled an occipital encephalocele. The true diagnosis was suspected only when his platelet count dropped to 3,000/cu mm with severe intestinal hemorrhage on the third day of life. The diagnosis was confirmed by arteriography. The hemangioma was excised surgically, and the platelet count returned quickly to normal. The baby has done well subsequently. When feasible, surgical excision of the platelet-trapping hemangioma is the treatment of choice in the hemangioma thrombocytopenia syndrome.     

Use of interferon in a case of hepatic hemangioma]

Hepatic hemangioendothelioma can have a fatal outcome. After failure of classic therapies, and when surgery or arterial embolization are not possible, those benign tumours may benefit from interferon alpha treatment. CASE REPORT: We report a case of a 14-month-old infant who presented with hepatic hemangiomatosis and cardiac failure. The disease can neither be controlled by steroids nor by radiotherapy associated with digitalo-diuretic treatment. Due to the important vascular volume of the tumour, neither surgical care nor arterial embolization were possible. Thus this infant was treated for ten months by interferon alpha. The evolution was favourable: cardiac failure, calcifications and volume of the angiomatosis were regressive. CONCLUSION: Interferon alpha treatment helps to control cardiac failure and the course of hepatic hemangioma in childhood.     

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。