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.     

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.     

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.     

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。     

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.     

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.     

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。     

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.     

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.     

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.     

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.     

Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with α-2A interferon

A two-month-old infant developed a vascular tumor of the right flank which, upon biopsy, proved to be a spindle cell hemangioendothelioma. The increased capillary bed characterizing the neoplasm caused a severe thrombocytopenia together with a consumption coagulopathy (Kasabach-Merritt syndrome). The patient, who was dependent on platelet transfusions, improved quickly after interferon α-2a was given at the dosage of 3,000,000 U/m², with resolution of the Kasabach-Merritt syndrome after three weeks and a 75% decrease of the tumor volume within three months of treatment. Med. Pediatr. Oncol. 28:358–361, 1997. © 1997 Wiley-Liss, Inc.     

Kasabach-Merritt综合征研究进展

Kasabach-Merritt综合征是以巨大血管肿瘤伴发血小板减少和全身出血倾向为特征的一种综合征,其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.治疗方案主要包括纠正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.     

Successful management with interferon alpha-2a after prednisone therapy failure in an infant with a giant cavernous hemangioma

A giant cavernous hemangioma of the left arm with severe thrombocytopenia and consumptive coagulopathy was observed in a neonate. Initial treatment with prednisone, platelet transfusions, and clotting replacement failed to control the bleedings. The child was then treated with daily subcutaneous infusions of interferon alpha-2a. Coagulopathy rapidly improved and transfusions were drastically reduced. The hemangioma regressed progressively and disappeared after 4 months of treatment. Med. Pediatr. Oncol. 28:213–215 © 1997 Wiley-Liss, Inc.     

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.     

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.     

Kasabach-Merritt syndrome: therapeutic considerations

During the past 15 years we have managed six children with capillary hemangiomas in association with consumptive coagulopathy--the Kasabach-Merritt syndrome. Their ages when first seen ranged from 2 weeks to 4 years with a mean of 19 months. In three of the patients, the hemangiomas remained small for many months and then suddenly enlarged and hemorrhagic diatheses appeared. The duration of the thrombocytopenia ranged from 5 to 20 months, a time span that in some of the patients was influenced by the type of treatment used. Each patient received a variety of therapies including the following: medications to control the coagulopathy; mechanical, cytolytic, and pharmacologic treatment to eradicate the lesion; and blood product support with platelets and cryoprecipitate for severe bleeding. A prompt elevation of the platelet count occurred in three of the patients after the lesion was biopsied. Subtotal resection of the lesion resulted in an immediate increase of the platelet count in one patient. A transient increase in platelet counts and fibrinogen levels was observed in another patient following several embolizations of a portion of the hemangioma. Other modes of therapy were difficult to evaluate, especially because the same therapy applied at different times in the same patient effected a different type of response. Eventually, all of the patients experienced resolution of their lesions and, with this, concomitant reversal of the coagulopathy.