Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico / Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach-Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach-Merritt, con excelente respuesta y tolerancia al propranolol

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach-Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

Evaluation and comparison of thromboelastography and conventional coagulation tests for blood coagulation function in children with Kasabach-Merritt syndrome / 中国输血杂志

【Objective】 To evaluate the coagulation function of children with Kasabach-Merritt syndrome(KMS)by thromboelastography (TEG) and conventional coagulation tests (CCTs), and to explore the correlation and consistency of the 2 test methods. 【Methods】 A total of 49 children with KMS, submitted to our hospital from January 2016 to December 2020, were enrolled. The TEG, CCTs data and platelet count were analyzed to evaluate the coagulation function, and the superiority of the 2 test methods were compared by Spearman correlation and Kappa consistency analysis. 【Results】 TEG and CCTs showed that the coagulation reaction time(R) was normal, the counts and function of platelet and fibrinogen decreased, and the D-dimer increased. The coagulation complex index (CI) indicated that the whole coagulation function was low. There was no significant difference in coagulation by sex or age in KMS children. The correlation analysis of TEG and CCTs in the coagulation function of KMS children showed that R was correlated with prothrombin time (PT) and activated partial thromboplastin Time(APTT), respectively (P<0.01); Fib had weak correlation with clot formation time (k)(r2=0.33), but strongly correlated with α-angle and MA value(r2=0.7, 0.69), respectively (P<0.01). PLT was moderately correlated with MA(r2=0.49, P<0.05); D-dimer had no correlation with LY30. Comparision resu lts of the consistency of TEG and CCTs showed that FIB and MA had consistency ( kappa=1, P<0.01); None or weak consistency was noticed among other indicators, R with PT/APTT, the kappa was 0.18 and 0.19; Fib with K/α-Angle, the kappa was 0.28 and 0.34; D-dimer with LY30, the kappa was 0.01; PLT with MA, the kappa was 0.35. 【Conclusion】 The main manifestations in low coagulation function in children with KMS were mainly thrombocytopenia, lower fibrinogen, and increased fibrinogen degradation-products, and the coagulation factors were normal. Except for Fib and MA, the consistency of other indexes in the detection of coagulation function in children with KMS by TEG and CCT is weak. Some indexes are significantly correlated but others not. Therefore, the 2 test methods are irreplaceable and should be combined to reduce the risk of embolism and bleeding in children.

Hemangioma esplênico no adulto associado à síndrome de Kasabach-Merritt / Kasabach-Merritt Syndrome-associated adult splenic hemangioma

O hemangioma esplênico, tumor benigno mais frequente do baço, porém, por vezes de difícil diagnóstico, pode integrar a síndrome de Kasabach-Merritt, afecção rara e potencialmente fatal, descrita como associação entre hemangioma, trombocitopenia e coagulopatia de consumo. Apresentamos um caso de paciente de 73 anos em acompanhamento com a hematologia por trombocitopenia crônica a princípio idiopática, que evoluiu com dor abdominal, anemia e derrame pleural. Foi realizada tomografia computadorizada de abdome, que evidenciou massa esplênica sólida heterogênea. A paciente foi submetida a esplenectomia total videolaparoscópica e evoluiu com melhora da dor abdominal e normalização da série plaquetária, sem recorrência do derrame pleural após o procedimento. A raridade e a complexidade do caso, somadas à dificuldade diagnóstica e à abordagem terapêutica, foram fatores que corroboraram para a apresentação desse caso. (AU)

Splenic hemangioma, the most common, but sometimes hard to diagnose, benign tumor of the spleen may integrate Kasabach- Merritt syndrome, a rare and potentially fatal condition described as an association ofhemangioma, thrombocytopenia and consumption coagulopathy. We present a case of a 73-year-old female patient being monitoring, with Hematology due to chronic idiopathic thrombocytopenia who progressed with abdominal pain, anemia and pleural effusion; a computed tomography scan of the abdomen was performed, showing a heterogeneous solid splenic mass. The patient underwent total videolaparoscopic splenectomy and progressed with improvement of abdominal pain and normalization of the platelet series, with no recurrence of pleural effusion after the procedure. The rarity and complexity of the case added to the diagnostic difficulty and therapeutic approach were factors that corroborated for the presentation of this case. (AU)

Hemangioma cavernomatoso hepático gigante, presentación infrecuente de una lesión común / Giant hepatic hemangioma, an uncommon presentation of a frequent disease

Los hemangiomas hepáticos, también denominados hemangiomas cavernomatosos, son los tumores hepáticos más comunes. Se caracterizan por ser lesiones solitarias, pequeñas y benignas que se diagnostican frecuentemente de forma incidental. Suelen ser lesiones asintomáticas, si bien los síntomas se presentan más frecuentemente en aquellas lesiones mayores de 5 cm, también conocidas como hemangiomas gigantes. Presentamos el caso de una mujer de 43 años, pauci-asintomática que presenta un hemangioma gigante de 16x16x27 cm, así como se realiza una revisión sistemática de la literatura.

Hepatic hemangiomas, also referred to as cavernous hemangiomas, are the most common benign mesenchymal hepatic tumors. They are often solitary, small lesions that have an excellent safety-prognosis and were commonly incidentally detected. Hepatic hemangiomas are frequently asymptomatic, although symptoms are more likely in those lesions larger than 5 cm also referred to as giant hemangiomas. We present a case of a pauci-asymptomatic 43 year-old woman with an uncommon 16x16x27 cm giant hemangioma and perform a review of the literature.

Anesthetic management of pediatric patients with Kasabach-Merritt phenomenon undergoing radical resection for huge hemangioma of head and neck / 中华麻醉学杂志

Nine pediatric patients (4 females,5 males) with huge hemangioma of head and neck complicated with Kasabach-Merritt phenomenon,aged 15-135 days,undergoing elective radical resection for huge hemangioma of head and neck,were selected from June 2012 to June 2016 in our hospital.Two pediatric patients were sensitive to preoperative hormone treatment,the platelet count almost increased to the normal value,7 pediatric patients were not sensitive to preoperative hormone treatment,and the increase in platelet count was not obvious.When the platelet count ＜ 40× 109/L,platelet was infused at 12 h before operation until the platelet count ＞ 100× 109/L.Two pediatric patients with larger haemangioma in maxillofacial region kept spontaneous breathing,and anesthesia was induced by inhaling high-concentration of sevoflurane.Anesthesia was induced with intravenous midazolam,sufentanil and cisatracurium in the other seven pediatric patients.Pediatric patients were mechanically ventilated in pressure-controlled mode after endotracheal intubation with airway pressure of 9-12 cmH2O.All pediatric patients inhaled sevoflurane,and anesthesia was maintained by infusing remifentanil.Heart rate and systolic blood pressure were maintained within the normal range during operation.Fluid and blood products were infused according to the blood loss,urine volume,physiological requirement and central venous pressure,etc.Pediatric patients were transferred to pediatric intensive care unit (PICU) at the end of operation,and the endotracheal tube was removed after the patients were completely awake.One pediatric patient developed pulmonary infection after operation,was discharged from PICU on day 7 after operation,then cured and discharged from hospital after healing on day 20 after operation.The other eight pediatric patients were discharged from PICU on day 2 after operation,then cured and discharged from hospital on days 5-10 after operation.After a followup period of 1 yr,the pediatric patients recovered well,the platelet count was normal,and the tumor recurrence was not found.

The angiographic characteristics and transcatheter arterial embolization therapy of Kasabach-Merritt syndrome / 中华放射学杂志

Objective To analyze the angiographic characteristics of Kasabach-Merritt syndrome (KMS) and study the value of transcatheter arterial embolization (TAE) in KMS therapy. Methods The clinical data of 36 infants with KMS treated between June 2010 and June 2016 in our hospital were reviewed retrospectively. All infants performed angiography and TAE therapy. These angiographic characteristics were analyzed by comparing with infantile hemangioma(IH).The times of TAE and the level of platelet after TAE therapy were observed. Results The angiographic characteristics of KMS were as follows: The capillary blush of KMS were ill defined with no-uniform distribution. There were a lot of very fine feeding arteries. The diameter of feeding artery was disproportionate to the volume of tumor blush because the feeding arteries were too small or fine. Normal artery was usually embedded in tumor blush. However, the angiographic characteristics of IH were different as follows:the capillary blush of IH were well defined with uniform distribution.There were about 1-4 feeding arteries.The diameter of feeding artery was proportional to the volume of tumor blush. Normal artery was not embedded in tumor blush. Tumor blush was usually located beside the normal artery. In 36 patients, 107 embolization treatments were performed. The platelet declined for 11 times and increased for 96 times after TAE therapy. The platelet count for these 96 cases increased to ≥100×109/L at(7.1 ± 2.4)days following therapy. However, the platelet level fluctuated thereafter and the average relapse time was(45.9 ± 21.8)days. All cases were followed-up ranging from 6 months to 2 years and finally received other therapies.Eight cases were cured and the other 28 cases were improved. Conclusions The angiographic characteristics of KMS are different from common infantile hemangioma. It is difficult to embolize the feeding arteries of KMS because there are a lot of very fine feeding arteries.TAE may rapidly improve the level of platelets while the long term effect was poor.It might be better to combine TAE with other medical therapies to treat KMS.

Refractory kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: clinical analysis of 10 cases / 中华儿科杂志

Objective@#To analyze the clinical value of sirolimus plus prednisone for the treatment of the refractory kaposiform hemangioendothelioma(RKHE) and Kasabach-Merritt syndrome(KMS).@*Method@#Clinical retrospective analysis was carried out for 10 patients recruited in Children′s Hospital Affiliated to Capital Institute of Paediatrics from January 2014 to January 2017 who were non responders to or relapsers after the treatment of propranolol, prednisone, pingyangmycin and lauromacrogol(5 cases RKHE, 5 cases RKHE plus KMS, age ranged from 6 days to 9 years); patients were treated with sirolimus at the dosage of 0.035 ml/(kg·d), once a day, for 6-410 days; the diagnosis of 10 patients were confirmed by pathological biopsy and immunohistochemical examination(IHC); the difference of the coagulation parameters and the platelet counts, the size of tumor and ecchymosis at different stages of treatment were recorded and measured by scale and ultrasonography; the side effects of sirolimus were recorded as well.@*Result@#Clinical characteristics of 10 cases (6 male and 4 female) RKHE with KMS were refractory dark red hard hemangioma or ecchymosis, the platelet counts were lower than 30.0×109/L, (15±7)×109/L, coagulation tests were obviously abnormal, fibrinogens were significantly decreased(0.8±0.5)g/L, the fibrin lysates and D-dimer were significantly increased(100±23)mg/L, (10 000±2 200)ng/L, the prothrombin time and activated partial thromboplastin time were prolonged(25.0±2.1)s, (58.0±3.4)s. The pathologic characteristics of the tumors were similar: spindle tumor cells, mass distribution and deeply stained nuclei tumor cells. IHC revealed positive staining for D2-40, CD31 and CD34. Stainings for factor Ⅷ and GLUT-1 were negative. In five cases RKHE plus KMS were treated with sirolimus and prednisone, after (6.5±0.7) days treatment, the platelet counts were obviously increased(72.0±0.6)×109/L, coagulation parameters were obviously improved, fibrinogen significantly increased(1.5±0.2)g/L, the fibrinlysates and D-dimer significantly decreased(7±3)mg/L, (2 300±200)ng/L, the prothrombin time and activated partial thromboplastin time were prolonged(15±2.3)s, (42±3.4) s, and the sizes of tumor and ecchymosis were slightly shrunken 18%±3%, 38%±5%; after (30±5.7) days treatment, the platelet counts and coagulation parameters returned to normal(146±36)×109/L, and the size of tumor and ecchymosis were obviously shrunken 73%±3%, 97%±3%; after (3±0.4) months treatment, the tumor was obviously shrunken by 93%±2% and no longer palpable. In five cases with RKHE without KMS manifested stubborn dark red hard hemangiomatous plaques, coagulation tests and platelet were obviously normal, these patients were treated with sirolimus, after (2.0±0.6) months treatment, the tumor became shrunken 8%±3%, with continuous treatment the tumor shrunk gradually, after (4.0±3.2)months(2-18 mouths) the tumor was not eliminated 51%±7%.@*Conclusion@#RKHE and KMS have typical clinical, laboratory and pathological characteristics, sirolimus plus prednisone have remarkable efficacy and minor side effects, it should be recommended for the treatment of KHE with KMS.

Perioperative nursing for children with Kasabach-Merritt syndrome undergoing transcatheter arterial scleroembolization / 现代临床护理

Objective To summarize the key points for nursing children with Kasabach-Merritt syndrome (KMS) treated by transcatheter arterial scleroembolization (TASE) during perioperative period. Method The perioperative treatment and key nursing points for 39 children with KMS undergoing TASE from September 2013 to September 2015 in our hospital were summarized and analyzed retrospectively. Results About 39 children with KMS went through TASE successfully. The operational time ranged from 0.8 to 2.7 hours. The patients were discharged in 2~11 days after TASE. There was 1 case of thrush, 3 cases of respiratory infection symptoms before TASE and 2 cases of hemangioma ulcer after TASE, which were cured by treatment and nursing. Conclusion The careful care to the patients with angeioma lesions, prevention and nursing of hemorrhage, nursing during glucocorticoid treatment, strict observation on the disease condition and prevention of complications after TASE can ensure the smooth implementation of TASE, and promote their recovery from KMS.

Treatment of refractory Kasabach-Merritt syndrome with transcatheter arterial embolization plus sirolimus therapy / 中华放射学杂志

Objective To investigate the efficacy and safety of transcather arterial embolization (TAE) plus sirolimus for the treatment of refractory Kasabach-Merritt syndrome (KMS) in infants. Methods Clinical data of twelve infants with refractory KMS treated between December 2015 and October 2016 in a single hospital were retrospectively analyzed. TAE were performed in all patients after failed traditional multiple therapies, followed by oral sirolimus administration. The dose of sirolimus was modulated according to the level of sirolimus, the count of platelet, the shrinkage of the lesion and the side effects, which were monitored regularly during the study. Results All 12 patients were treated with TAE plus sirolimus therapies successfully. The platelet count for all patients increased to≥100×109/L for the first time at (7±5) days. Stabilization of platelet level was obtained in (15±7) days averagely. Before the treatment, two infants had a normal fibrinogen level and the fibrinogen level in the other 10 infants was found to be increased to≥2.0 g/L at (9 ± 4)days for the first time and was then stabilized at levels>2.0 g/L at (19 ± 7)days after treatment. One patient showed skin fester (GradeⅡ), one patient had a fever with acute pulmonary infection (Grade Ⅲ) and both patients improved well after symptomatic treatment. There were no serious complications in the other ten patients. Conclusions TAE plus sirolimus can rapidly improve levels of platelets and fibrinogen, and it is a safe, useful and effective method for treatment of refractory KMS in infants.

Perioperative nursing for children with Kasabach-Merritt syndrome undergoing transcatheter arterial scleroembolization / 现代临床护理

Objective To summarize the key points for nursing children with Kasabach-Merritt syndrome (KMS) treated by transcatheter arterial scleroembolization (TASE) during perioperative period. Method The perioperative treatment and key nursing points for 39 children with KMS undergoing TASE from September 2013 to September 2015 in our hospital were summarized and analyzed retrospectively. Results About 39 children with KMS went through TASE successfully. The operational time ranged from 0.8 to 2.7 hours. The patients were discharged in 2~11 days after TASE. There was 1 case of thrush, 3 cases of respiratory infection symptoms before TASE and 2 cases of hemangioma ulcer after TASE, which were cured by treatment and nursing. Conclusion The careful care to the patients with angeioma lesions, prevention and nursing of hemorrhage, nursing during glucocorticoid treatment, strict observation on the disease condition and prevention of complications after TASE can ensure the smooth implementation of TASE, and promote their recovery from KMS.

Treatment of refractory Kasabach-Merritt syndrome with transcatheter arterial embolization plus sirolimus therapy / 中华放射学杂志

Objective To investigate the efficacy and safety of transcather arterial embolization (TAE) plus sirolimus for the treatment of refractory Kasabach-Merritt syndrome (KMS) in infants. Methods Clinical data of twelve infants with refractory KMS treated between December 2015 and October 2016 in a single hospital were retrospectively analyzed. TAE were performed in all patients after failed traditional multiple therapies, followed by oral sirolimus administration. The dose of sirolimus was modulated according to the level of sirolimus, the count of platelet, the shrinkage of the lesion and the side effects, which were monitored regularly during the study. Results All 12 patients were treated with TAE plus sirolimus therapies successfully. The platelet count for all patients increased to≥100×109/L for the first time at (7±5) days. Stabilization of platelet level was obtained in (15±7) days averagely. Before the treatment, two infants had a normal fibrinogen level and the fibrinogen level in the other 10 infants was found to be increased to≥2.0 g/L at (9 ± 4)days for the first time and was then stabilized at levels>2.0 g/L at (19 ± 7)days after treatment. One patient showed skin fester (GradeⅡ), one patient had a fever with acute pulmonary infection (Grade Ⅲ) and both patients improved well after symptomatic treatment. There were no serious complications in the other ten patients. Conclusions TAE plus sirolimus can rapidly improve levels of platelets and fibrinogen, and it is a safe, useful and effective method for treatment of refractory KMS in infants.

Hemangioblastoma cerebeloso y trombocitopenia: reporte de caso / Cerebellar hemangioblastoma and thrombocytopenia: report of one case

The association between vascular tumors and thrombocytopenia is rare. Kasabach-Merritt Syndrome is seen in childhood and is characterized by hemangiomas and thrombocytopenia. A 42 years-old man with a cerebellar hemangioblastoma and thrombocytopenia, admitted with a subarachnoid hemorrhage is reported. The patient was operated and required a splenectomy to manage the thrombocytopenia. After the splenectomy the patient developed a subdural hematoma that was operated. Despite the surgical treatment, the patient died.

Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience

BACKGROUND: Kasabach-Merritt syndrome (KMS) is a rare but life-threatening illness. The purpose of this study is to report our single-center experience with KMS. METHODS: We reviewed the medical records of 13 patients who were diagnosed with KMS between 1997 and 2012 at Samsung Medical Center. Treatment response was defined as follows: 1) hematologic complete response (HCR) – platelet count >130×10⁹/L without transfusion; 2) clinical complete response (CCR) – complete tumor disappearance or small residual vascular tumor displaying lack of proliferation for at least 6 months after treatment discontinuation. RESULTS: Participants included 7 male and 6 female patients. The median initial hemoglobin levels and platelet counts were 9.7 g/dL (range, 6.6–11.6 g/dL) and 11×10⁹/L (range, 3–38×10⁹/L), respectively. Twelve patients received corticosteroid and interferon-alpha as initial treatment, and the remaining patient received propranolol instead of corticosteroid. Two patients with unsatisfactory response to the initial treatment received weekly vincristine. Successful discontinuation of medication was possible at a median of 301 days (range, 137–579) in all patients except one who was lost to follow-up. The median times to achieve HCR and CCR were 157 days and 332 days, respectively. The probabilities of achieving HCR and CCR were 77% and 54% at 1 year, and 88% and 86% at 2.5 years, respectively. CONCLUSION: The prognosis of KMS in our cohort was excellent. Our data suggest that individualized treatment adaptation according to response may be very important for the successful treatment of patients with KMS.

Hepatic Hemangioma with Kasabach-Merritt Syndrome in an Adult Patient / 대한소화기학회지

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.

A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol / 임상소아혈액종양

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.

A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol / 임상소아혈액종양

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.

Interventional approaches to neonatal Kasabach-Merritt syndrome：method selection and therapeutic effectiveness / 中华放射学杂志

Objective To explore therapeutic effects of different interventional approaches to neonatal Kasabach-Merritt syndrome (K-MS) Methods A retrospective analysis of therapeutic effects of different interventional approaches to K-MS in 12 neonates from May 2010 to August 2014. All the patients received local treatment (n=5), chemoembolization (n=4) and combined local treatment and endovascular chemoembolization (n=3), respectively, depending on size, location and blood supply of K-MS. The K-MS patients with angioma measuring5 cm received local treatment, endovascular chemoembolization and combined local treatment and endovascular chemoembolization, respectively. The patients were categorized as cured, improved and clinical inefficient, respectively, based on therapeutic effect. The criteria for cured patients were: (1) angioma faded completely or mostly; (2) the platelet count stabilized within the normal range; (3)no recurrence for more than six months. The criteria for improved patients were: (1) angioma shrinked significantly; (2) the platelet stabilized within the normal range. The criteria for clinical inefficient patients were: (1) agioma no obviously shrinked, the platelet count has no improvement over 2 months after interventional approaches. Results All the 12 patients with K-MS were cured and had no complication. Of the 12 patients, 5 patients received local treatment had decreased localized tension and ecchymosis of hemangioma at the first day of therapy, were cured after 2 to 7 courses of therapy. Of the 4 patients received endovascular chemoembolization, 3 were cured after one course of treatment, 1 had recurrent disease one month later after one course of therapy and were cured after the second course of treatment.One had recurrent disease a month later after one course of treatment and were cured after the second course of treatment. Three received combined local treatment and endovascular chemoembolization, angioma shrinked significantly and increased platelet count after the first course of treatment, and 2 to 3 courses local treatment were combined, hemangioma faded and platelet count stabilized within the normal range after 2 to 3 courses of local treatment. Conclusions Interventional therapy is the most effective treatment of neonatal K-MS.There are different interventional approaches to optimize treatment of neonatal K-MS depending on sizes, location and blood supply of K-MS.

Kasabach-Merritt syndrome: clinical vs. surgical treatment / Síndrome de Kasabach-Merritt: tratamento clínico versus cirúrgico

Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient's clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article.

A síndrome de Kassabach-Merritt é uma associação de hemangioma capilar e trombocitopenia, que promove sangramentos com petéquias, equimoses e hematomas espontâneos. A conduta é tratar com corticoide, interferon alfa ou quimioterápicos. Apresentamos um caso de criança (com 1 ano e 9 meses anos de idade) com um hemangioma gigante desde a raiz da coxa até altura do joelho, e plaquetopenia. O tratamento foi iniciado com corticoterapia sem melhora do quadro, surgindo de forma espontânea pontos de sangramento intratumoral e cutâneo. Sem condições clínicas de receber vincristina e o interferon, foi feita a ressecção tumoral de urgência por extrema plaquetopenia e sangramento. A criança começou então a desenvolver um quadro séptico com hipotensão e isquemia do tecido residual. Diante deste quadro, configurou-se um desafio terapêutico que será objeto deste artigo.

Clinicopathological analysis of a case of adult Kasabach-Merritt syndrome / 中华皮肤科杂志

A 52-year-old female presented with unexplained thrombocytopenia for 29 years and a prunosus plaque on the right cheek for 9 years.She had ever been treated with glucocorticoids,which resulted in a slight improvement of the condition,but the platelet count remained below the normal range.During glucocorticoid tapering,she had administrated traditional Chinese medicines for a long peroid.Recently,the plaque on the cheek became swollen and painful with a progressive decrease in platelet count.Routine blood test showed that the count of white blood cells and platelets was 3.0 × 109/L and 2 × 109/L respectively,and the concentration of hemoglobin was 85 g/L.Computed tomography (CT) scan revealed multiple liver hemangiomas,one of which in the left liver lobe measured 14 cm × 5.7 cm in size.After medical treatment,the giant hemangioma in the left lobe was resected.Thereafter,the platelet count increased to 120 × 109/L with the subsidence of swelling of the right cheek.Postoperative pathological examination confirmed a diagnosis of giant cavernous hemangioma of the liver with extensive intravascular thrombosis and thrombus organization.Based on these findings,the case was diagnosed as adult Kasabach-Merritt syndrome.

Clinical analysis of 17 cases of neonatal Kasabach-Merritt syndrome / 国际儿科学杂志

Objective To summarize the clinical characteristics and therapeutic approaches of Kasabach-Merritt syndrome (KMS).Methods The data from 17 KMS patients that had been definitely diagnosed from December,2007 to January,2012 in our tertiary center were collected retrospectively.Results We described 17 patients,13 of whom were male and 4 of whom were female,with an age range of 17 hours to 28 days.Of the 17 cases,4 cases had hemangioma of internal organs,13 on the surface of the body.All of them had thrombocytopenia and coagulation dysfunction.Seventeen cases were initially treated with corticosteroids.The responses were varied:excellent and rapid improvement (n =6),failure (n =11),recurrence (n =3).Then the cases of failure and recurrence accepted artery embolization:excellent and rapid improvement (n =8),failure (n =4),recurrence (n =1),with 1 giving up.At last,the cases resistant to artery embolization therapy accepted vincristine therapy.Four cases had rapid improvement,and 1 died from disseminated intravascular coagulation.In this study,a response rate to corticosteroids was 35.3％,and the recurrent rate was 50％.The response rate to artery embolization was 61.5％.Five patients unresponsive to hormone therapy and artery embolism were treated with vincristine,and the effective rate was 80％.Conclusion In the therapy of neonatal KMS,the resistant to corticosteroids is common.Combinative therapy of corticosteroids with artery embolization is recommended as the first-line therapy.Vincristine is suggested as a therapy when there is resistance to the other therapy.