-Neonatal Kasabach-Merritt syndrome healed by complete surgical excision of the angioma-.

BACKGROUND: Kasabach-Merritt syndrome is an infrequent combination of a giant hemangioma and severe thrombocytopenia. This syndrome may be life-threatening. There are various but no definitive methods of treatment. We describe here a neonatal case cured by total excision of the tumor. CASE REPORT: A boy was admitted at birth with a giant hemangioma of the back with thrombocytopenia. Tumor compression, corticosteroids, acetylsalicylic acid and ticlopidine were ineffective. Total surgical excision was performed on day 51, resulting in complete recovery. CONCLUSION: Total surgical excision of a localized hemangioma in a non-functional area is probably the most appropriate and justified treatment for the Kasabach-Merritt syndrome.     

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。     

Radiotherapy in the management of Kasabach-Merritt syndrome: a case report

A 21-day-old infant with hemangioma was brought to the hospital with enlargement of the hemangioma of the left thigh and purple discoloration around umbilicus. This led to the diagnosis of Kasabach-Merritt syndrome. Initial treatment with corticosteroid failed. The patient under went radiotherapy that led success fully to improvement of both hemangioma and thrombocytopenia. After 2 years of follow-up, the patient has experienced long-term complications, including atrophy and growth retardation of the irradiated leg.     

RADIOTHERAPY IN THE MANAGEMENT OF KASABACH-MERRITT SYNDROME: A Case Report

A 21-day-old infant with hemangioma was brought to the hospital with enlargement of the hemangioma of the left thigh and purple discoloration around umbilicus. This led to the diagnosis of Kasabach-Merritt syndrome. Initial treatment with corticosteroid failed. The patient under went radiotherapy that led success fully to improvement of both hemangioma and thrombocytopenia. After 2 years of follow-up, the patient has experienced long-term complications, including atrophy and growth retardation of the irradiated leg.     

Successful Treatment of Kasabach-Merritt Syndrome with Prednisone and Epsilon-Aminocaproic Acid

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.     

The Kasabach-Merritt syndrome with pancreatic localization in a newborn infant

The case of a neonate with a large pancreatic hemangioma belonging to the Kasabach-Merritt syndrome is reported. Selective embolization of one of the main vessels originating from the gastroduodenal artery resulted in a progressive increase of blood platelet levels and in a concomitant change in the hemangiomatous structure at ultrasound examination, as well as in a subsequent acceleration of somatic growth. At 8 months of age, there were no more clinical, radiological, ultrasonographic or biological signs of the hemangioma. This case report emphasizes the interest of embolization in the Kasabach-Merritt syndrome.     

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.     

The Kasabach-Merritt syndrome: treatment with intermittent pneumatic compression.

A 6 week old infant presented with a giant angiomatous naevus of the leg complicated by chronic disseminated intravascular coagulation. The bleeding and laboratory abnormalities responded to intermittent pneumatic compression of the affected limb. This innocuous treatment should be considered in the Kasabach-Merritt syndrome.     

A case of axillary kaposiform hemangioendothelioma resembles a soft tissue sarcoma

Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It is often complicated with Kasabach-Merritt syndrome. The authors describe a 2-month-old girl who developed a rapidly enlarging deep subcutaneous solid tumor in the left axilla. The tumor size was about 10x7 cm and there was no skin change over the mass. The results of laboratory tests were significant for a moderate anemia (7.0 g/dL) and a profound thrombocytopenia (3x10/L). The preoperative radiologic findings were suspicious for a soft tissue sarcoma with scapular erosion. Anemia and thrombocytopenia were corrected by preoperative corticosteroid therapy and blood component transfusion. The tumor was excised totally and the pathologic findings were consistent with Kaposiform hemangioendothelioma. Serial blood examinations showed that the hemoglobin and platelet count returned to the normal range after surgery.     

Tranexamic acid in the treatment of Kasabach-Merritt syndrome in infants

Kasabach-Merritt syndrome (thrombocytopenia, consumption coagulopathy, and, occasionally, microangiopathic hemolysis) is an infrequent but often lethal complication of giant capillary hemangiomas in the infant. We report the clinical course of an infant who came to us with this syndrome shortly after birth. She was successfully treated with transfusions of blood products, high-dose oral prednisone, and a fibrinolytic inhibitor, tranexamic acid. No complications of the treatment occurred. The hematological abnormalities resolved completely by 2 years of age. Although prednisone may have been necessary for stabilization and involution of the hemangioma, the addition of tranexamic acid allowed earlier tapering of the steroid therapy and fewer transfusions. Tranexamic acid was extremely well tolerated in this newborn.     

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.     

Development of Kasabach-Merritt syndrome following needle aspiration of a hemangioma.

A case of Kasabach-Merritt syndrome that developed following needle aspiration in an infant with hemangioma is presented. Enlargement of the tumor leading to severe respiratory distress accompanied by hemorrhages occurred within six to eight hours after this simple intervention. The symptoms continued despite repeated thrombocyte transfusions and prednisolone therapy. Radiotherapy and local intraarterial corticosteroid therapy were attempted and led to some improvement. It took approximately three months for the platelet count and one year for the clinical state to revert to normal. This case illustrates that minor trauma may lead to life-threatening symptoms in a young infant with congenital hemangioma.     

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。     

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。     

Kaposiform hemangioendothelioma associated with Kasabach-Merritt syndrome

OBJECTIVE: To describe a case of kaposiform hemangioendothelioma, the only malignant tumor of vascular origin specific of childhood.METHODS: We report a case of a 40-days-old girl who presented with a giant hemangioma of the face. Rapid enlargement of the tumor lead to laryngeal compression with severe respiratory distress. She had also a consumptive thrombocytopenic coagulopathy (Kasabach-Merritt syndrome).RESULTS: She was admitted to the pediatric intensive care unit of the Hospital das Clínicas da Universidade Estadual de Campinas and mechanical ventilation was introduced. A ten day course of dexamethasone was only partially effective. We then started alpha-2a interferon at a dose of 1.8 million units/m(2)/day by subcutaneous route, but she died four days after the onset of this therapy. The microscopic features showed a kaposiform hemangioendothelioma.CONCLUSION: We discuss this unusual fatal evolution of a rapidly growing hemangioma and its hematological complications.     

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.     

Hydrops Fetalis Caused by Fetal Kasabach-Merritt Syndrome

There have been only 2 previous reports of nonimmunologic hydrops fetalis (NIHF) caused by fetal Kasabach-Merritt syndrome, both of which were pathological studies. This is the first clinical case report of NIHF due to fetal Kasabach-Merritt syndrome that was prenatally diagnosed by sonography, computerized tomography, and percutaneous umbilical blood sampling.     

Kasabach-Merritt综合征治疗中自体原位植皮术的应用

目的 对Kasabach-Merritt综合征手术治疗过程中使用自体原位植皮术,评价其治疗效果,提出一种新的手术治疗方式.方法 回顾性分析2010年至2015年间自体原位植皮手术治疗的Kasabach-Merritt综合征患儿23例,病例纳入标准:均为瘤体分布弥散,皮肤大面积(＞5 cm×5 cm)异常,手术切除瘤体后创面缝合困难;非手术治疗方法无效;血小板值在2×109/L～31×109/L.23例Kasabach-Merritt综合征患儿瘤体均位于四肢,上肢6例,下肢17例;男9例,女14例;年龄7 d～9个月.总结该手术治疗方法的手术要点及术中、术后处理,分析治愈率及术后并发症.术后均经病理学检查证实为卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)20例,丛状血管瘤(tufted angioma,TA)3例.结果 23例患儿均在术后1周内血小板值恢复正常,随访1～5年,治愈率为100％;18例患儿自体皮片原位移植全部成活,有3例成活面积约90％,经后续换药处理后愈合,2例原位皮片移植成活约60％,行二期皮肤移植修补手术后治愈,术后并发症少.结论 手术治疗Kasabach-Merritt综合征可作为有效的治疗方法,本组资料显示,自体原位植皮术作为一种新的手术方式,治愈率100％,术后并发症少,其对符合适应证者是一种可选择治疗手段.     

Stepwise multimodal approach in the treatment of Kasabach–Merritt syndrome

BACKGROUND: The purpose of the present study was to evalutate the various treatment modalities for Kasabach-Merritt syndrome (KMS) and to identify the most reliable treatment modalities. METHODS: A retrospective analysis was performed on 37 KMS patients who were admitted to Seoul National University Hospital between January 1979 and June 1999. Age, sex, locations of the hemangiomas, clinical symptoms and hematologic data were analyzed by multivariate logistical regression analysis to determine the response to various treatment modalities. RESULTS: Twenty-four of 37 patients (20 boys and 17 girls) were diagnosed during infancy. The locations of hemangioma were superficial skin in 31 patients, combined skin and visceral organs in four patients and visceral organs only in two patients. Tumor size was more than 5 cm in diameter in all cases, except for one. The treatment principle of KMS in our center involves a stepwise multimodal approach. Of the 37 cases, surgical resection was performed in two. Steroids were tried initially in 35 patients over a 1 week period, with an initial response rate of 11.4% (4/35). Combined steroid/radiation treatment was given in 28 cases, with a response rate of 75.0% (21/28). Four of five patients with additional interferon (IFN)-alpha therapy improved. No significant correlations were found between the prognostic factors, such as age, sex, size and locations of the hemangioma, hematologic data and the treatment modalities. CONCLUSIONS: Based on the experiences in a single center over a period of 20 years, we recommend a stepwise multimodal approach for the treatment of KMS; for example, steroid, radiation therapy and IFN-alpha in this order. However, surgical treatment is helpful if total resection is possible.     

激素抵抗型婴幼儿Kasabach-Merritt综合征治疗中雷帕霉素的临床应用价值探讨

目的 初步评估临床上应用雷帕霉素治疗激素抵抗型婴幼儿Kasabach-Merritt综合征的临床疗效及安全性.方法 2015年2月至2016年4月共收集8例在我科接受治疗的Kasabach-Merritt综合征患儿,其中男5例,女3例,病灶位于颌面部3例、颈部1例、四肢2例,胸腹壁2例,8例患儿均表现为激素抵抗,对激素抵抗型Kasabach-Merritt综合征采用mTOR抑制剂雷帕霉素治疗.雷帕霉素服用方法为每次0.8 mg/m2,2次/d,间隔12h,血药浓度维持10～15 ng/ml.定期监测血常规、凝血功能、肝肾功能、血脂及雷帕霉素血药浓度等指标.根据血药浓度、血小板变化、凝血功能、瘤体缩小情况及副作用可适当调整用药计划.结果 8例患儿经雷帕霉素治疗后血小板均恢复正常,瘤体萎缩,并逐步撤离了激素,有效率达到100％.雷帕霉素平均起效时间(6.8±2.7)d,平均血小板稳定时间(19.1±8.5)d,目前雷帕霉素总的用药时间为4～10个月,平均(6.0±2.2)个月,开始雷帕霉素单药治疗时间为2～8个月,平均(4.5±1.9)个月.8例患儿仍在服药进行中,均无血小板下降和病灶复发.药物使用中不良反应主要为口腔黏膜炎及口腔溃疡(2例,GradeⅡ)、呕吐(1例,Grade工)、腹泻(2例,Grade Ⅰ/GradeⅡ)、发热(2例,GradeⅡ)、皮疹(1例,Grade Ⅰ)、疼痛(1例,Grade Ⅰ)、短暂性转氨酶及血脂异常(1例,Grade Ⅰ/GradeⅡ),予对症治疗后好转,无严重不良事件,无病例退出.结论 mTOR抑制剂雷帕霉素治疗激素抵抗型Kasabach-Merritt综合征具有一定的疗效及安全性,值得临床进一步推广应用.