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    1 OF 13

    A case of an elderly female with diffuse hepatic hemangiomatosis complicated with multiple organic dysfunction and Kasabach-Merritt syndrome.
    Clin J Gastroenterol 2018 May 29. Epub 2018 May 29.
    Department of Gastroenterology, National Hospital Organization Kanazawa Medical Center, 1-1 Shimoishibikicho, Kanazawa, Ishikawa, 920-8650, Japan.
    Since diffuse hepatic hemangiomatosis (DHH) is an extremely rare disease especially in adults, the etiology and natural course of adult-onset DHH has not been well understood. We report a case of DHH complicated with multiple organic dysfunction and Kasabach-Merritt syndrome (KMS) in an 83-year-old female. She presented with mild abdominal distension and laboratory findings revealed thrombocytopenia and abnormal coagulation, indicating disseminated intravascular coagulation (DIC). Read More

    [Benign aggressive vascular anomalies in children].
    Bull Cancer 2018 Jun 20;105(6):610-625. Epub 2018 Mar 20.
    Centre de référence des maladies rares de la peau d'origine génétique, hôpital Pellegrin-Enfants, centre hospitalier universitaire de Bordeaux, unité de dermatologie pédiatrique, 33076 Bordeaux cedex, France.
    Superficial vascular anomalies constitute a large group of malformative and tumoral conditions developed from all types of vessels. Vascular tumors are the result of cellular hyperplasia, whereas vascular malformations (VMs) are constituted of dysplastic vessels. The classification from International Society for the Study of Vascular Anomalies (ISSVA) is based on this pathogenic difference. Read More

    Clinical and imaging features of Kaposiform Hemangioendothelioma.
    Br J Radiol 2018 Jun 20;91(1086):20170798. Epub 2018 Mar 20.
    3 Department of Radiology, Fudan University Shanghai Cancer Center , Shanghai , China.
    Objective: Kaposiform hemangioendothelioma (KHE) is a unique locally aggressive vascular tumor with poor prognosis. The aim of this study is to assess the clinical and imaging features of KHE, and to compare the differences between solitary and diffusive infiltrative subtype further.

    Methods: The clinical and radiological findings of a cohort of 25 cases with histologically proven KHE, between June 2011 and June 2016, were reviewed retrospectively. Read More

    Kasabach-Merritt syndrome combined with hypercalcemia: A case report.
    Exp Ther Med 2017 Dec 18;14(6):6164-6168. Epub 2017 Oct 18.
    Department of Hematology and Oncology of Children's Medical Center, Hunan Provincial People's Hospital, Changsha, Hunan 410005, P.R. China.
    The present case report presented the diagnosis and treatment course of an infant diagnosed with Kasabach-Merritt syndrome (KMS) combined with hypercalcemia (HC). A 35-day-old infant with swelling on the upper right arm for >1 month and thrombocytopenia for 1 day was admitted to Hunan Provincial People's Hospital (Changsha, China) and a series of treatments, including γ-globulin impact, heparin anticoagulation, platelet transfusion, supplement of cryoprecipitate and fibrinogen following heparinization and inhabitation of vascular endothelial cell proliferation by propranolol, were performed. At 2 months after the initial admission to the hospital, surgery was conducted and the hemangioma was removed through pipeline arteriosclerosis embolization when the patient was hospitalized again with symptoms of vomiting and atrophy accompanied by HC. Read More

    Association between extremity kaposiform hemangioendothelioma and lymphedema.
    Pediatr Dermatol 2018 Jan 20;35(1):e92-e93. Epub 2017 Dec 20.
    Department of Plastic and Oral Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.
    Kaposiform hemangioendotheliomas are pediatric vascular tumors that do not metastasize. We present a patient with a thigh kaposiform hemangioendothelioma successfully treated using a systemic corticosteroid during infancy who was diagnosed with lymphedema in the extremity 9 years later. The observation that extremity kaposiform hemangioendothelioma could possibly be associated with lymphedema has implications for the care of patients with kaposiform hemangioendothelioma. Read More

    Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin.
    J Pediatr Hematol Oncol 2018 May;40(4):e239-e242
    Division of Hematology.
    Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a syndrome of platelet trapping known as Kasabach-Merritt phenomenon that, through deposition of platelet derived growth factors, may perpetuate the growth of the tumor. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often refractory even to aggressive treatment. Herein, we describe a patient with a refractory, recurrent KHE despite multiple attempts at local control and intensive chemotherapy, that ultimately was successfully treated with rationally designed and low-intensity combination therapy of sirolimus and aspirin. Read More

    [Vascular anomalies. Part I: vascular tumors].
    Chirurg 2018 Mar;89(3):245-254
    Klinik und Poliklinik für Gefäßchirurgie und Endovaskuläre Chirurgie, Universitätsklinikum, Universität zu Köln, Kerpener Str. 62, 50937, Köln, Deutschland.
    Vascular anomalies represent a spectrum of structural disorders and inborn errors of vascular morphogenesis, which may affect the entire arterial and venous vascular tree. They are divided into two major categories: tumors, which exhibit endothelial hyperplasia and malformations, which have normal endothelial turnover unless disturbed. Depending on their nature and complexity they can range from a simple "birthmark" to life-threatening entities. Read More

    Infantile hemangiomas: a 7-year experience of a single-center.
    Clujul Med 2017 20;90(4):396-400. Epub 2017 Oct 20.
    Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
    Objectives: The aim of the study was to describe the historical and clinical characteristics of hemangiomas in a series of cases of our clinic.

    Methods: This is a retrospective study of 36 patients with infantile hemangiomas consulted in our clinic.

    Results: We had 14 multiple hemangiomas, and 1 kaposiform hemangioendothelioma. Read More

    Propranolol in the preoperative treatment of Kasabach-Merritt syndrome: a case report.
    J Med Case Rep 2017 Oct 27;11(1):308. Epub 2017 Oct 27.
    Pediatric Clinic, Institute for Childhood Diseases, Clinical Centre of Montenegro, Podgorica, Montenegro.
    Background: Kasabach-Merritt syndrome represents the association of hemangioma with thrombocytopenia and consumptive coagulopathy. We present a case of Kasabach-Merritt syndrome treatment with orally administered propranolol.

    Case Presentation: A 4. Read More

    Intestinal obstruction due to kaposiform hemangioendothelioma in a 1-month-old infant: A case report.
    Medicine (Baltimore) 2017 Sep;96(37):e6974
    Department of Pediatric Surgery, Seoul National University College of Medicine, Seoul, Korea.
    Rationale: Kaposiform hemangioendothelioma (KHE) is an aggressive vascular tumor, mainly occurring in infants and young children and previously reported cases were mainly cutaneous or visceral form. Intestinal kaposiform hemangioma was first reported in 2012. Intestinal type KHE showed better prognosis if the lesion was limited in the gastrointestinal tract and coagulopathy was not accompanied. Read More

    [Refractory kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: clinical analysis of 10 cases].
    Zhonghua Er Ke Za Zhi 2017 Sep;55(9):700-704
    Department of Neurology, Children's Hospital Affiliated to Capital Institute of Paediatrics, Beijing 100020, China.
    To analyze the clinical value of sirolimus plus prednisone for the treatment of the refractory kaposiform hemangioendothelioma(RKHE) and Kasabach-Merritt syndrome(KMS). 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. Read More

    [Acute complications of vascular anomalies in childhood].
    Hautarzt 2017 Oct;68(10):790-795
    Abt. Pädiatrische Dermatologie/Allergologie, Kath. Kinderkrankenhaus Wilhelmstift, Liliencronstr. 130, 22149, Hamburg, Deutschland.
    Congenital and acquired vascular anomalies are common in childhood. In addition to predominantly harmless vascular skin alterations there are others which must be immediately treated due to the potentially threatening complications. As examples three anomalies and the typical complications are presented. Read More

    Successful combination therapy of propranolol and prednisolone for a case with congenital Kasabach-Merritt syndrome.
    J Dermatol 2017 Dec 3;44(12):1389-1391. Epub 2017 Aug 3.
    Department of Dermatology, Faculty of Medicine, Mie University, Tsu, Japan.
    A male fetus was delivered by cesarean section with a large hemangioma on his right chest and thrombocytopenia. Clinically, Kasabach-Merritt syndrome (KMS) was suspected, and immediately he was treated with daily prednisolone (PSL) 1 mg/kg and recombinant thrombomodulin without response. Additional propranolol (1-3 mg/kg per day) and increased PSL 2 mg/kg per day therapy successfully controlled his disseminated intravascular coagulation and decreased the tumor size without serious side-effects. Read More

    Giant liver hemangioma with adult Kasabach-Merritt syndrome: Case report and literature review.
    Medicine (Baltimore) 2017 Aug;96(31):e7688
    Department of Hepatobiliary Surgery, China-Japan Friendship Hospital, Beijing, China.
    Rationale: Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is rare; to date, most reports have been single-case reports, and no multi-case reports or literature reviews are available.

    Diagnoses: We conducted a retrospective analysis of 5 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma treated at our hospital between 2011 and 2016. All 5 patients had varying severities of leukopenia, anemia, thrombocytopenia, prolonged prothrombin time, and hypofibrinogenemia. Read More

    A Huge Subcutaneous Hematoma in an Adult with Kasabach-Merritt Syndrome.
    Am J Case Rep 2017 Jun 19;18:682-686. Epub 2017 Jun 19.
    Department of Medical Imaging, Changhua Christian Hospital, Changhua, Taiwan.
    BACKGROUND Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh. Read More

    Multimodal treatment of Kasabach-Merritt syndrome arising from tufted angioma: A case report.
    Oncol Lett 2017 Jun 20;13(6):4887-4891. Epub 2017 Apr 20.
    Department of Reconstructive Plastic Surgery, The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, P.R. China.
    Kasabach-Merritt syndrome (KMS) is a rare type of vascular tumor associated with a severely decreased platelet count. No standard guidelines for the treatment of the disease have been established so far. In the present study, a 1-year-old pediatric patient with KMS arising from tufted angioma was successfully and variously treated with steroids, vincristine, surgery and propranolol for 18 months. Read More

    Proposal of size-based surgical indication criteria for liver hemangioma based on a nationwide survey in Japan.
    J Hepatobiliary Pancreat Sci 2017 Jul 22;24(7):417-425. Epub 2017 Jun 22.
    2nd Department of Surgery, Wakayama Medical University, Wakayama, Japan.
    Background: Surgical indications for liver hemangioma remain unclear.

    Methods: Of 510 patients undergoing surgical resection for liver hemangioma in 118 Japanese centers between 1998 and 2012, abdominal symptoms, diagnostic accuracy, and surgical outcomes were analyzed to propose size-based surgical indications. Patients were classified into four groups based on tumor size: Group A ≤5 cm (n = 122, 24%), Group B 5-10 cm (n = 164, 32%), Group C 10-15 cm (n = 124, 24%), and Group D >15 cm (n = 100, 20%). Read More

    Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study.
    Int J Cancer 2017 08 26;141(4):848-855. Epub 2017 May 26.
    Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA.
    Kaposiform hemangioendothelioma (KHE) is an aggressive disease with high morbidity and mortality. The aim of this study was to retrospectively evaluate the efficacy and safety of sirolimus for the treatment of progressive KHE. A multicenter, retrospective cohort study was conducted in patients with progressive KHE treated with sirolimus. Read More

    Kaposiform haemangioendothelioma: a review with emphasis on histological differential diagnosis.
    Pathology 2017 Jun 21;49(4):356-362. Epub 2017 Apr 21.
    Department of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
    Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive/borderline vascular tumour primarily seen in neonates and children. KHE is occasionally associated with Kasabach-Merritt phenomenon and tends to have a poor clinical prognosis. While the histological features of tufted angiomas and KHE overlap, some believe tufted angiomas are a milder, benign, more localised counterpart of KHE. Read More

    Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome.
    World J Gastroenterol 2017 Apr;23(13):2443-2447
    Sanya Wadhwa, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, United States.
    Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. Read More

    Extremely Giant Liver Hemangioma (50 cm) with Kasabach-Merritt Syndrome.
    J Gastrointest Surg 2017 Oct 19;21(10):1748-1749. Epub 2017 Apr 19.
    Department of Hepatobiliary Surgery, China-Japan Friendship Hospital, 2 Yinghua Dongjie, Hepingli, Beijing, 100029, China.
    A 33-year-old male has been found with a giant liver hemangioma of initial size 29 cm for 5 years. He received arterial embolization twice in order to shrink the tumor; however, no effect was obtained. The tumor had rapidly grown to 50 cm and caused abnormalities in the hematological and coagulative systems. Read More

    Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma.
    Pediatr Dermatol 2017 May 6;34(3):e146-e149. Epub 2017 Apr 6.
    Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee.
    Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that does not metastasize. We report two unique cases of KHE involving the right hemithorax and the upper ipsilateral extremity. Kasabach-Merrit phenomenon and osteolytic lesions in the scapula were observed in both cases. Read More

    Kaposiform Hemangioendothelioma Presenting as Hydrops Fetalis.
    Pediatr Dermatol 2017 May 20;34(3):e128-e129. Epub 2017 Mar 20.
    Section of Neonatology, Department of Pediatrics, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
    We describe the case of a 33-week preterm infant who developed nonimmune hydrops fetalis secondary to a kaposiform hemangioendothelioma (KHE). The tumor was successfully treated with vincristine, prednisone, ticlopidine, and aspirin. KHE can be an unusual cause of hydrops fetalis; in such cases, diagnosis can be challenging since generalized edema can obscure KHE. Read More

    A New Perspective for Infantile Hepatic Hemangioma in the Age of Propranolol: Experience at Baskent University.
    Exp Clin Transplant 2017 Mar;15(Suppl 2):74-78
    Department of Pediatric Oncology-Hematology, Baskent University Faculty of Medicine, Ankara, Turkey.
    Propranolol was first used in 2008 to treat hemangioma; its efficacy and safety have since changed the classical treatment indications. Infantile hepatic hemangioma presents as a spectrum of clinical conditions varying from simple asymptomatic lesions to lethal complications. Tufted hemangioma and Kaposiform hemangioendothelioma are congenital vascular tumors that lead to Kasabach-Merritt syndrome. Read More

    [Role of master transcriptional factor Prox-1 in lymphatic endothelial differentiation of Kaposiform hemangioendothelioma].
    Zhonghua Bing Li Xue Za Zhi 2017 Mar;46(3):176-181
    Department of Pathology, Xijing Hospital, Fourth Military Medical University, Xi'an 710032, China.
    To analyze the clinical and pathological features of Kaposiform hemangioendothelioma (KHE), and to investigate the role of master transcriptional factor Prox-1 in the regulation of lymphatic differentiation. Nine cases of KHE (during the period from October 2009 to June 2016) were collected with clinical and pathological data. H&E stained section review and immunohistochemietry using the Dako EnVision method were performed. Read More

    Sirolimus for Vincristine-Resistant Kasabach-Merritt Phenomenon: Report of Eight Patients.
    Pediatr Dermatol 2017 May 15;34(3):261-265. Epub 2017 Feb 15.
    Department of Pediatric Surgery, Second Affiliated Hospital, College of Medicine, Xi'an JiaoTong University, Xi'an, China.
    Background: The use of sirolimus for patients with multidrug-resistant Kasabach-Merritt phenomenon (KMP) has been reported in recent years. We present the experience of a single center in treating vincristine-resistant KMP using sirolimus alone.

    Methods: Children with vincristine-resistant KMP who were treated with oral sirolimus alone were eligible for inclusion in the study. Read More

    [Myelophthisis and kasabach merrit syndrome as initial manifestation of splenic angiosarcoma].
    Rev Fac Cien Med Univ Nac Cordoba 2016 ;73(4):297-301
    Primary splenic angiosarcoma is an extremely agressive and rare neoplasm. Manifestations as bone marrow invasion and coagulation disorders have been reported isolatedly. A 26 years-old woman presented with abdominal pain; several anemia and thrombocytopenia associated to leukoerythroblastic reaction were found in the laboratory. Read More

    Characteristics and operative treatment of extremely giant liver hemangioma >20 cm.
    Surgery 2017 06 23;161(6):1514-1524. Epub 2017 Jan 23.
    Department of Hepatobiliary Surgery, China-Japan Friendship Hospital, Beijing, China.
    Background: Giant liver hemangioma >20 cm may cause severe complications; therefore, operative treatment can be highly difficult and risky. No studies have been performed to determine the characteristics of this subgroup.

    Methods: A retrospective study was performed on 141 patients who underwent operative treatment for liver hemangioma. Read More

    Successful surgical management of congenital Kasabach-Merritt syndrome.
    Pediatr Int 2017 Jan;59(1):89-92
    Pediatric Plastic Surgery Department, Timone Enfants University Hospital, Marseille, France.
    Since the first description of Kasabach-Merritt syndrome (KMS) in 1940, many treatments have been proposed combining pharmacologic and non-pharmacologic approaches, which can be effective on the pathology but can have adverse and unpredictable side-effects with long-term use. Herein we describe the solely surgical treatment of Kasabach-Merritt syndrome in a neonate with a severe and rapidly progressive thrombocytopenia. The patient's condition normalized at 7 days postoperatively, with rapid increase in platelet count and normalization of d-dimer concentration. Read More

    Exome sequence analysis of Kaposiform hemangioendothelioma: identification of putative driver mutations.
    An Bras Dermatol 2016 Nov-Dec;91(6):748-753
    Faculty of Life Sciences, Kumamoto University - Kumamoto, Japan.
    Background:: Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments.

    Objective:: In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. Read More

    Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience.
    Blood Res 2016 Dec 23;51(4):256-260. Epub 2016 Dec 23.
    Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.; Department of Medical Device Management and Research, SAIHST, Sungkyunkwan University, Seoul, Korea.; Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, Korea.
    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. Read More

    Imaging findings of Kaposiform Hemangioendothelioma in children.
    Eur J Radiol 2017 Jan 10;86:198-205. Epub 2016 Nov 10.
    Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea.
    Purpose: Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Though several case reports have discussed the imaging findings of KHE, larger comprehensive studies are lacking. The purpose of this study was to evaluate the imaging findings of KHE in children. Read More

    Multifocal Kaposiform Hemangioendothelioma Causing Massive Fetal Chylous Ascites.
    Fetal Pediatr Pathol 2017 Apr 16;36(2):162-167. Epub 2016 Dec 16.
    b Department of Obstetrics and Gynecology , College of Medicine, Inje University Busan Paik Hospital , Busan , Republic of Korea.
    Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that usually occurs in soft tissues of the extremity and rarely in the retroperitoneum. We report a unique case of isolated massive fetal ascites attributed to KHE, involving the retroperitoneum and multiple visceral organs, along with the Kasabach-Merritt phenomenon. We suspect that retroperitoneal KHE might have caused massive fetal ascites because of its high potential to invade the lymphatic vessels aggressively in the retroperitoneal space, which possibly permits intestinal lymph leakage into the peritoneal cavities. Read More

    Awareness of primary spontaneous hemorrhagic angiosarcoma of the breast associated with Kasabach-Merritt syndrome in a pregnant woman by enhanced magnetic resonance imaging: A CARE-compliant case report.
    Medicine (Baltimore) 2016 Nov;95(44):e5276
    aDepartment of Diagnostic Radiology, Chang Gung Memorial Hospital, ChiayibDepartment of Medical Imaging and Intervention, Chang Gung Memorial Hospital, LinkoucDepartment of Medical Imaging and Intervention, Chang Gung Memorial Hospital, KeelungdDepartment of PathologyeDepartment of Surgery, Chang Gung Memorial Hospital, LinkoufMedical College of Chang Gung University, Taiwan.
    Introduction: Primary breast angiosarcoma with spontaneous intratumoral bleeding associating with Kasabach-Merritt Syndrome is rarely reported.

    Case Findings/patient Concerns: We herein present such a case in a 30-year-old pregnant woman who was initially diagnosed to hemangioma at her early gestation. However, the sudden rapid tumor growth was aware of the attention and intended for receiving the breast enhanced magnetic resonance imaging. Read More

    Anesthestic management of Kassabach-Meritt Phenomenon in an adult: case report.
    BMC Anesthesiol 2016 11 9;16(1):109. Epub 2016 Nov 9.
    Department of Anesthesiology and Intensive Care, Military Hospital Mohamed V of Rabat, Faculty of Medicine and Pharmacy of Rabat, University Mohamed V-Souissi, Rabat, Morocco.
    Background: Kasabach-Merritt phenomenon (KMP) is characterized by a vascular tumor with profound thrombocytopenia and consumptive coagulopathy that may presents significant challenges for anesthesiologist.

    Case Presentation: An 87-year-old man presented with kaposiform hemangioendothelioma involving the right leg in critical condition due to massive bleeding. Hematology investigations indicated the presence of KMP. Read More

    [Unexpected cutaneous purpura in an infant].
    Zhongguo Dang Dai Er Ke Za Zhi 2016 Nov;18(11):1154-1157
    Department of Dermatology, Hunan Children's Hospital, Changsha 410007, China.
    A two-month-old boy visited the hospital due to unexpected cutaneous purpura and thrombocytopenia for 2 days. The physical examination revealed a purple mass on the back. The soft tissue color Doppler ultrasound showed rich blood signals in the tissue, and the results of bone marrow puncture indicated an increased number of megakaryocytes. Read More

    Successful low-dose radiotherapy treatment for Kasabach-Merritt syndrome.
    Pediatr Int 2016 Oct;58(10):1084-1086
    Department of Pediatrics, Showa University Fujigaoka Hospital, Yokohama, Japan.
    Kasabach-Merritt syndrome (KMS) is characterized by hemangioma associated with life-threatening thrombocytopenia, and is a consumptive coagulopathy. Although treatments available include corticosteroids, α-interferon, vincristine, and surgery, response may be unsatisfactory, and the mortality rate remains at approximately 30%. Although radiotherapy has been used effectively for KMS, it may cause growth retardation and secondary malignancy. Read More

    Interferon-alpha therapy for refractory kaposiform hemangioendothelioma: a single-center experience.
    Sci Rep 2016 10 31;6:36261. Epub 2016 Oct 31.
    Department of Oral and Maxillofacial Surgery, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai 200011, China.
    Kaposiform hemangioendothelioma (KHE) is a relatively rare vascular tumor with an aggressive and infiltrating nature. Previous studies have revealed an exclusive relationship between KHE and Kasabach-Merritt Phenomenon (KMP), which is associated with high morbidity and mortality. No universally accepted treatment modality exists for refractory KHE with or without KMP. Read More

    Successful Everolimus Treatment of Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon: Clinical Efficacy and Adverse Effects of mTOR Inhibitor Therapy.
    J Pediatr Hematol Oncol 2016 11;38(8):e322-e325
    *Department of Pediatrics, Gifu Prefectural General Medical Center Departments of †Pediatrics ‡Orthopedic Surgery ∥Pathology, Gifu University Graduate School of Medicine, Gifu University, Gifu §Department of Pediatrics and Cell Transplantation, Mie University Graduate School of Medicine, Tsu, Japan.
    Kasabach-Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy associated with underlying kaposiform hemangioendothelioma (KHE) in infancy. We describe the case of a 3-month-old girl with KHE complicated by KMP who responded dramatically to treatment with everolimus, a mechanistic target of rapamycin (mTOR) inhibitor. Immunohistochemical expression of mTOR was found in the KHE biopsy specimens, which may explain the improvement of KMP and reduction in KHE tumor size with mTOR inhibitor treatment. Read More

    Sirolimus in the Treatment of Vascular Anomalies.
    Eur J Pediatr Surg 2017 Feb 10;27(1):86-90. Epub 2016 Oct 10.
    Department of Plastic Pediatric Surgery, Hospital La Paz, Madrid, Spain.
     mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Read More

    Long-term outcome for kaposiform hemangioendothelioma: A report of two cases.
    Pediatr Blood Cancer 2017 Feb 4;64(2):284-286. Epub 2016 Oct 4.
    Department of Pediatric Hematology/Oncology, Boston Children's Hospital, Boston, Massachusetts.
    Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life-threatening coagulopathy known as Kasabach-Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. Read More

    The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma.
    Semin Cutan Med Surg 2016 Sep;35(3):147-52
    Department of Pediatrics, Division of Dermatology, Seattle Children's Hospital/University of Washington School of Medicine, Seattle, Washington, USA.
    Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy. Read More

    A Rare Presentation of Isolated Congenital Splenic Hemangioma With Kasabach-Merritt Syndrome.
    J Pediatr Hematol Oncol 2017 Jan;39(1):e29-e32
    Department of Pediatrics, Division of Neonatology, University of Miami, Holtz Children's Hospital, Miami, FL.
    Introduction: Splenic hemangiomas (SHs) are the most common benign neoplasms of the spleen. However, they are rare in the newborn period. We present an extremely rare case of congenital SH complicated by Kasabach-Merritt syndrome. Read More

    Lymphoedema is a potential sequela of Kaposiform haemangioendothelioma: reply from the authors.
    Br J Dermatol 2016 10 11;175(4):834. Epub 2016 Aug 11.
    Department of Dermatology and Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), Université Paris Descartes - Sorbonne Paris Cité, Institut Imagine, Hôpital Universitaire Necker-Enfants Malades, 149 Rue de Sèvres, 75015, Paris, France.

    Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood.
    Indian J Pathol Microbiol 2016 Jul-Sep;59(3):392-4
    Department of Pathology, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India.
    Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach-Merritt syndrome. Read More

    [Cerebellar hemangioblastoma and thrombocytopenia: Report of one case].
    Rev Med Chil 2016 Apr;144(4):521-5
    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. Read More

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