Kaposiform hemangioendothelioma in an unusual site: A report of two cases in children

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor usually seen in children. It is frequently associated with Kasabach-Merritt phenomenon. Here we report two cases of KHE: the first case being an 11-month-old boy who came with massive swelling on the face and violaceous discoloration. The second case was a 7-year-old boy who presented with respiratory distress and bleeding manifestations. CT scan chest showed a large mass involving the anterior mediastinum. Histologic examination of resected masses from both these cases showed features of KHE involving subcutaneous tissue and thymus, respectively. Although cutaneous and subcutaneous location is common, thymic involvement is unusual. It is important to distinguish KHE from infantile haemangioma, tufted angioma, spindle-cell haemangioma, verrucous malformation and Kaposi sarcoma. Histologic features, supportive immunohistochemistry and the clinical profile together are helpful to differentiate KHE from other vascular lesions.

SARS-CoV-2 precipitated Kasabach-Merritt syndrome in a child with angioma infantile and acute lymphoblastic leukemia: Case Report / Síndrome de Kasabach-Merritt precipitado por SARS-CoV-2 en un niño con angioma infantil y leucemia linfoblástica aguda: Reporte de Caso

SUMMARY Background: We describe an 8-month-old boy with leukemia and SARS-CoV-2 infection who developed Kasabach-Merritt phenomenon. He had a positive SARS-COV-2 RT-PCR sample. Hematologic tests showed coagulopathy and intestinal involvement. She was managed in emergency receiving transfusion support and in hospitalization with social isolation measures, she started propanolol and corticotherapy as initial treatment of infantile angiomas. She presented with symptoms of intestinal obstruction and underwent surgery and evidence of hemorrhagic infarction with foci of intestinal ischemic necrosis, ending in ileostomy. We tried to understand a pathophysiological explanation of the dermatologic and gastrointestinal tract involvement by the virus and the atypical form of COVID-19. Given the emerging evidence of endothelial and vascular involvement in COVID-19, the development of tests to detect vascular lesions may be critical to guide the use of new therapeutic strategies.

RESUMEN Introducción: a un niño de 8 meses con leucemia e infección por SARS-CoV-2 que desarrolló el fenómeno de Kasabach-Merritt. Tenía una muestra de RT-PCR de SARS-COV-2 positiva. Las pruebas hematológicas mostraron coagulopatía y afectación intestinal. Fue manejada en emergencia recibiendo apoyo transfusional y en hospitalización con medidas de aislamiento social, inició propanolol y corticoterapia como tratamiento inicial de los angiomas infantiles. Presentó síntomas de obstrucción intestinal y fue intervenida quirúrgicamente y se evidenció infarto hemorrágico con focos de necrosis isquémica intestinal, terminando en ileostomía. Intentamos comprender una explicación fisiopatológica de la afectación dermatológica y del tracto gastrointestinal por el virus y la forma atípica de COVID-19. Dada la evidencia emergente de la afectación endotelial y vascular en COVID-19, el desarrollo de pruebas para detectar lesiones vasculares puede ser crítico para guiar el uso de nuevas estrategias terapéuticas.

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura / Refractory kaposiforme hemangioendothelioma in the pediatric population: case report and literature review

Resumen Introducción: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente caracterizado por una invasión local agresiva y un síndrome de atrapamiento de plaquetas conocido como fenómeno de Kasabach-Merritt. Aunque muchos casos de HEK se tratan con éxito con control local o quimioterapia de baja intensidad, otros son resistentes y se cuenta con pocas opciones terapéuticas. El objetivo de este reporte es mostrar la experiencia del tratamiento con sirolimus por vía oral en un paciente pediátrico con HEK asociado a fenómeno de Kasabach-Merritt refractario al tratamiento de primera línea, quien mostró excelente respuesta al tratamiento. Caso clínico: Paciente de sexo masculino de 3 meses con un HEK refractario al manejo de primera línea (corticoides, propranolol, vincristina), sin posibilidad de hacer control local, por lo que se decide terapia combinada con sirolimus, presentando control local y resolución de la coagulopatía desde la primera semana de iniciado el manejo y con resolución de la malformación vascular después de 12 meses de seguimiento. Conclusiones: Aunque no existen pautas claras para el tratamiento del HEK refractario en la edad pediátrica, la evidencia actual demuestra que el sirolimus es un medicamento eficaz que puede ser considerado como opción terapéutica de primera línea en estos pacientes.

Abstract Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a platelet entrapment syndrome known as the Kasabach-Merritt phenomenon. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often resistant, with few therapeutic options available. Here, we report a pediatric patient with KHE associated with Kasabach-Merritt phenomenon refractory to first-line treatment, who demonstrated excellent response to treatment. Case report: We present the case of a 3-month-old male patient with a KHE refractory to first-line treatment (vincristine, corticosteroids, propranolol), without possibility of local control treatment. Therefore, combined therapy with sirolimus was decided, presenting local control and resolution of the coagulopathy from the first week after starting the management and with resolution of vascular malformation after 12 months of follow-up. Conclusions: Although there are no clear guidelines for the treatment of refractory KHE in the pediatric population, current evidence demonstrate that sirolimus is an effective option that could be considered as a first-line treatment in such patients.

Síndrome de Kasabach-Merritt esplénico en adulto. Presentación de un caso y revisión del tema / Splenic Kasabach-Merritt syndrome in an adult. Presentation of a case and review of the topic / Síndrome de Kasabach-Merritt esplênica em adulto. Apresentação de um caso e revisão do tema

RESUMEN Se presentó un paciente de 22 años de edad, masculino, de la raza negra con gran esplenomegalia debido a un hemangioma gigante del bazo, el cual presentó trombocitopenia y trastornos de la coagulación. Se estudió y se diagnosticó un síndrome de Kasabach-Merrit. Se le realizó una esplenectomía total anterior regresando a la normalidad el conteo de plaquetas y los trastornos de la coagulación. Su evolución fue favorable al cabo de los 18 meses de seguimiento. Se recomienda el tratamiento quirúrgico, la esplenectomía. Se revisó el tema por lo infrecuente de la enfermedad.

ABSTRACT A 22-year-old male black patient presented with big splenomegaly due to a giant hemangioma of the spleen, showing thrombocytopenia and bleeding disorders. A Kasabach-Merrit syndrome was diagnosed. An anterior total splenectomy was performed, returning the platelet count and coagulation disorders back to normal. His evolution was favorable after 18 months of follow-up. Splenectomy surgical treatment was recommended. The topic was reviewed due to the infrequency of the disease.

RESUMO Foi apresentado paciente do sexo masculino, 22 anos de idade, de raça negra, apresentou grande esplenomegalia devido a hemangioma gigante do baço, que apresentava plaquetopenia e distúrbios de coagulação. Uma síndrome de Kasabach-Merrit foi estudada e diagnosticada. Uma esplenectomia total anterior foi realizada, retornando a contagem de plaquetas e distúrbios de coagulação ao normal. Sua evolução foi favorável após 18 meses de seguimento. Tratamento cirúrgico é recomendado, esplenectomia. O tema foi revisado devido à raridade da doença.

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 hepático congénito gigante: síndrome de Kasabach-Merritt / Giant Congenital Liver Hemangioma: Kasabach Merritt Syndrome

Resumen Los hemangiomas son las proliferaciones más frecuentes en la edad pediátrica, y el hemangioma hepático es de los más comunes entre los viscerales. El diagnóstico precoz es importante para orientar el tratamiento. Se presenta el caso de un neonato con compromiso ventilatorio secundario al efecto de una masa gigante, que ecográficamente correspondió a un hemangioma hepático. Se realizó resección quirúrgica y, ulteriormente, fue enviada a patología, que confirmó el diagnóstico.

Abstract Hemangiomas are the most frequent proliferations in children, and liver hemangiomas are the most common among those in the viscera. Early diagnosis to focus treatment is important. We present the case of a neonate with compromised breathing secondary to the effect of a giant mass that echographically appeared to be a hepatic hemangioma. Surgical resection and subsequent pathology confirmed the diagnosis.

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.

Neonatal RICH associated with thrombocytopenia and coagulopathy: a case report and literature review / 中华整形外科杂志

We treated a neonate who was diagnosed as rapidly involuting congenital hemangioma (RICH). With a review on the clinical manifestation, differential diagnosis, and treatment of RICH that associated with thrombocytopenia(TP)and coagulopathy(CP)and received platelet transfusions and embolization therapy including absolute ethanol and polidocanol in our hospital in March of 2015. The platelets and coagulation function soon returned to normal, the tumor involuted significantly. Surgical excision was proceeded subsequently. The platelets returned to normal level after a one-time platelet transfusion, meanwhile, multiple reexaminations of blood coagulation function were normal. Postoperatively, incision wound healed well. There was no recurrence, and the functional recovery of upper limb was satisfactory. RICH is a rare type of vascular tumor which may present with TP and CP similar to KHE-KMP in the neonatal period. More attention should be addressed to identify these two diseases, because the treatment and prognosis of which have significant differences. Consumption of coagulation factors with milder platelet decrease may also be a complication of slow flow venous or venolymphatic malformations.

Perioperative platelet transfusion in infantile with Kasabach-Merritt phenomenon insensitive to glucocorticoids / 中华整形外科杂志

Objective@#To assess the safety and effectiveness of sufficient, short-term platelet (PLT) transfusion for the surgery preparation of the infantile patients with Kasabach-Merritt phenomenon, who were insensitive to glucocorticoids.@*Methods@#The infantile cases were retrospectively analyzed during May 2011 to December 2016, who were clinically diagnosed as KMP and insensitive to glucocorticoids, received PLT transfusion and surgical resection. PLT transfusion in patients whose PLTC was less than 30×109/L, was 0.3 therapeutic dose(TD)/kg, and 0.2 TD/kg in PLTC≥30×109/L group. The maximum was 1 TD.Criteria of the PLT transrusion: 1 hour after the transfusion, the PLT count (PLTC) were tested and the corrected count increment of platelet (CCI) and practical platelet recovery (PPR) was calculated. PLTC ≥100×109/L, CCI>7.5×109/L and PPR>30% were defined as effective; while PLTC=(50-99)×109/L, CCI>7.5×109/L and PPR>30% as partial effective; PLTC<50×109/L, or CCI≤7.5×109/L, or PPR ≤30% were defined as ineffective. By reviewing the method and response of their PLT infusions, to figure out the most effective way in rising PLT, as a part of pre-operation treatment.@*Results@#There were 46 cases in the research. Based on the PLTC, CCI and PPR 1 hour after PLT transfusion, there were 44 effective transfusion, 2 patients with partial effectiveness, and no ineffective case. There was no allergic or heart failure happened in any cases. No critical potential complications of PLT transfusion occurred, including fluid and iron overload, alloimmunization to human leukocyte antigen and/or PLT antigen.@*Conclusions@#Pre-operative sufficient and short-term PLT infusions are more effective than low dose and long-term ones. They can create a more optimistic opportunity for surgical resections.

Recent advances in the diagnosis and treatment of Kasabach-Merritt phenomenon / 国际儿科学杂志

Kasabach-Merritt phenomenon (KMP),also known as Kasabach-Merritt syndrome,is a rare syndrome associated with Kaposiform haemangioendothelioma or tufted angioma,and characterized by thrombocytopenia and consumptive coagulopathy.KMP onsets early and progresses quickly.If diagnosis and treatment delayed,it can be life-threatening.In this paper,the progress of clinical manifestations,pathogenesis,diagnosis and treatment of KMP are summarized.

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.

Kasabach-Merritt phenomenon in 9 neonates / 中国新生儿科杂志

Objective To study the clinical features,diagnosis and treatment in the neonates of Kasabach-Merritt phenomenn (KMP).Method To analyze retrospectively the data collected from the 9 neonates of Kasabach-Merritt phenomenon in our neonatal intensive care unit from January 2016 to March 2017.The data of their clinical presentation,imaging findings,treatment and outcomes were analyzed.Result Among the 9 cases,there were 6 males and 3 females,with onset age of 0 ～ 14 d.The hemangiomas were located over the body surface of the neck,back,trunk and limbs in 4 cases.The others were located in the deep organs in 5 cases.2 cases were found hepatic hemangiomas by fetal ultrasound and 3 cases had symptoms of poor response,dyspnea and nasal bleeding with occult onset.All the 9 cases had thrombocytopenia,and the average platelet count was 24.0 × 109/L.The imaging findings showed the changes of hemangiomas,and among them,3 cases were associated with arteriovenous fistula.In one case,the biopsy pathology diagnosis was Kaposiform hemangioendothelioma.Among 8 cases which was treated,2 cases had systemic medication,2 cases had interventional embolization and 4 cases were treated with combined therapy (interventional embolization together with medication),including 1 case of definitive surgical treatment.Finally 7 cases improved,1 case with severe thrombocytopenia and coagulation disorders did not improve and treatment abandoned and 1 case died before treatment.Conclusion The symptomatology of KMP is various,which is related to the location of the lesion.The imaging studies are helpful to diagnosis.Massive hemorrhage and multiple organ dysfunction can be fatal.The treatments are different among cases,for most cases with proper treatment the prognosis are good.

Retrospective analysis in 13 children with Kasabach-Merritt phenomenon and review of literature / 临床儿科杂志

Objective To improve understanding of the clinical manifestations, diagnosis and treatment of childhood Kasabach-Merritt phenomenon (KMP). Methods The clinical data of 13 patients admitted for KMP to XXX from January 2010 to January 2016 was retrospectively analyzed, with a review of relevant literature. Results The patients were 10 males and 3 females. The age of presentation varied from newborn to 5 months. 12 patients had cutaneous manifestations, like petechiae, ecchymosis, jaundice, skin masses, etc, 1 patient had pleural effusion. The location of lesions varied. The laboratory hallmark consists of profound thrombocytopenia and hypofibrinogenemia with elevated D-dimers. The median time from initial presentation to diagnosis was 60 days. After approaches like surgery, corticosteroids, propranolol, interferon, sirolimus, etc, 10 patients got remission while 3 patients died. 6 patients treated with sirolimushad complete response. Conclusions KMP is characterized with vascular tumor, severe thrombocytopenia and consumptive coagulopathy. Clinically, KMP often presents with early-onset and delay in diagnosis. Surgery is an effective approach for KMP. Sirolimus appears to be a promising treatment for KMP.

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.

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.

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.