707 results match your criteria Kasabach-Merritt Syndrome


Nonimmune hydrops fetalis: identifying the underlying genetic etiology.

Genet Med 2018 Nov 9. Epub 2018 Nov 9.

Department of Obstetrics, Gynecology, & Reproductive Sciences, University of California, San Francisco, CA, USA.

Purpose: Numerous etiologies may lead to nonimmune hydrops fetalis (NIHF), and the underlying cause often remains unclear. We aimed to determine the proportion of NIHF cases in which the etiology was clearly determined in a large, contemporary, and diverse cohort, as well as to describe the etiologies with a focus on genetic causes.

Methods: Retrospective review of NIHF cases between 2015 and 2017 from the five University of California Fetal-Maternal Consortium sites. Read More

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November 2018
1 Read

Kasabach-Merritt Syndrome in an Adult: A Comment.

Turk J Haematol 2018 Oct 22. Epub 2018 Oct 22.

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October 2018
1 Read

Tufted angioma arising at the site of hepatitis B vaccination: A case report.

Turk J Pediatr 2018 ;60(2):188-190

Molecular Dermatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Sepaskhah M, Hajizadeh J, Sari-Aslani F, Jowkar F. Tufted angioma arising at the site of hepatitis B vaccination: A case report. Turk J Pediatr 2018; 60: 188-190. Read More

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January 2018
12 Reads

A rapidly growing violaceous tumor in a neonate.

JAAD Case Rep 2018 Oct 3;4(9):893-895. Epub 2018 Oct 3.

Division of Dermatology, Medical College of Georgia at Augusta University, Augusta, Georgia.

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October 2018
1 Read

Kaposiform hemangioendothelioma without cutaneous involvement.

J Cancer Res Clin Oncol 2018 Dec 6;144(12):2475-2484. Epub 2018 Oct 6.

Department of Vascular and Interventional Radiology, Chengdu Women and Children's Central Hospital, Chengdu, 610031, China.

Purpose: We sought to characterize the clinical features and management of patients diagnosed as Kaposiform hemangioendothelioma (KHE) without cutaneous involvement.

Methods: The electronic patient chats at six Triple A hospitals in China were searched to find all patient diagnoses with KHE without cutaneous involvement.

Results: Of 30 patients (mean age at diagnosis, 55. Read More

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December 2018
2 Reads
3.080 Impact Factor

Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon: clinical characteristics and management.

Cancer Manag Res 2018 7;10:3325-3331. Epub 2018 Sep 7.

Department of Vascular and Interventional Radiology, Chengdu Women and Children's Central Hospital, Chengdu, China.

Purpose: Musculoskeletal complications have been associated with kaposiform hemangioendothelioma (KHE) and can lead to disability and reduced quality of life. We aimed to determine the clinical characteristics of musculoskeletal complication in patients with KHE without Kasabach-Merritt phenomenon (KMP) in order to identify features that may aid clinicians in KHE treatment.

Patients And Methods: We conducted a cohort study of KHE without KMP associated with musculoskeletal complication between January 2006 and February 2017 at three tertiary medical centers in China. Read More

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September 2018
1 Read

Development of Kasabach-Merritt phenomenon following vaccination: More than a coincidence?

J Dermatol 2018 Oct 17;45(10):1203-1206. Epub 2018 Aug 17.

Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.

Kasabach-Merritt phenomenon (KMP) occurred uniquely in patients with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). We report the clinical characteristics of two patients with KHE involving the right upper arm. The patients demonstrated rapid enlargement of the lesion with severe KMP shortly after vaccination. Read More

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October 2018
16 Reads

Retrospective study on the outcomes of infantile tufted angioma complicated by Kasabach-Merritt Phenomenon.

Clin Chim Acta 2018 Nov 7;486:199-204. Epub 2018 Aug 7.

Department of Hemangioma and Vascular Malformation Surgery, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, 7 Weiwu Road, Zhengzhou 450003, China. Electronic address:

Objective: To analyze the clinical characteristics and treatment of pediatric tufted angiomas(TA)complicated by Kasabach-Merritt Phenomenon (KMP).

Method: A retrospective analysis was conducted on the clinical data and follow-up data of 13 patients diagnosed with TA complicated by KMP. Five male and 8 female patients with an average age of 5. Read More

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November 2018
13 Reads

Efficacy of sirolimus in an infant with Kasabach-Merritt phenomenon.

Pediatr Int 2018 Sep 8;60(9):887-889. Epub 2018 Aug 8.

Department of Pediatrics, Gifu University Graduate School of Medicine, Yanagido, Japan.

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September 2018
1 Read

Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in an infant: Successful treatment with prednisolone, vincristine, and addition of sirolimus.

Pediatr Blood Cancer 2018 Dec 1;65(12):e27305. Epub 2018 Aug 1.

Department of Pediatric Hematology/Oncology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.

A full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach-Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. Read More

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December 2018
1 Read

First Intracardiac Kaposiform Hemangioendothelioma in an Infant Resolved With Sirolimus: A Case Report.

J Pediatr Hematol Oncol 2018 Oct;40(7):536-540

Department of Pediatric Cardiology, Advocate Children's Hospital, Oak Lawn.

Introduction: Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor that can be associated with a consumptive coagulopathy and thrombocytopenia (Kasabach-Merritt phenomenon). Only one case of an intracardiac KHE has been reported which was treated with surgical excision and then expectant management.

Case Presentation: We present a patient with an intracardiac KHE which presented as a large mass surrounding the atria, pulmonary veins, superior vena cava, and infiltrating the atrial septum with moderate compression of the superior vena cava and mild compression of the pulmonary veins. Read More

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October 2018
8 Reads

Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options.

World J Pediatr 2018 08 27;14(4):322-329. Epub 2018 Jul 27.

Pediatric Surgery, Dr. von Hauner Childen's Hospital, Ludwig-Maximilians University of Munich, Munich, Germany.

Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor affecting infants and young children. Although benign, it can be associated with an aggressive locally growing tumor and/or a life-threatening Kasabach-Merritt phenomenon (KMP). To date, only reviews of limited cases have been performed. Read More

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August 2018
10 Reads

Sirolimus Treatment of an Infant With Intrathoracic Kaposiform Hemangioendothelioma Complicated by Life-threatening Pleural and Pericardial Effusions.

J Pediatr Hematol Oncol 2018 Jul 23. Epub 2018 Jul 23.

Paediatrics, Division of Haematology/Oncology.

Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that may be associated with Kasabach-Merritt Phenomenon (KMP), which is a consumptive coagulopathy with potentially life-threatening thrombocytopenia. Management of KHE and KMP is challenging, and currently, there are no standardized validated treatment protocols. Mammalian target of rapamycin inhibitors have been shown to be effective in the treatment of KHE. Read More

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July 2018
1 Read
0.960 Impact Factor

Successful Management of Kaposiform Hemangioendothelioma with Long-Term Sirolimus Treatment: a Case Report and Review of the Literature.

Mediterr J Hematol Infect Dis 2018 1;10(1):e2018043. Epub 2018 Jul 1.

Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.

Background: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and the first decade of life. It is locally aggressive and potentially life threatening when associated with consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of prospective trials, and the different standard care suggestions are based on retrospective case series. Read More

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July 2018
5 Reads

Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma.

Pediatr Dermatol 2018 Sep 12;35(5):635-638. Epub 2018 Jul 12.

Department of Pediatric Surgery, Second Affiliated Hospital, Medical College of Xi'an, JiaoTong University, Xi'an, China.

Background: Sirolimus has been used to manage various complex vascular anomalies. Kaposiform hemangioendothelioma and tufted angioma may develop Kasabach-Merritt phenomenon in infancy.

Methods: We retrospectively reviewed the clinical and laboratory data of eight patients with kaposiform hemangioendothelioma and tufted angioma who were initially treated using oral sirolimus in our center, including six with Kasabach-Merritt phenomenon. Read More

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September 2018
2 Reads

Coexistence of Kasabach-Merritt Syndrome and placental chorioangioma in a premature infant.

J Neonatal Perinatal Med 2018 ;11(2):209-213

Kasabach-Merritt syndrome is a rare life-threatening clinical presentation in neonatal period. it is characterized by giant hemangioma and serious thrombocytopenia. The diagnostic criteria include: 1) hemangiomas on skin, 2) thrombocytopenia or coagulopathy, 3) hemangioma on internal organs diagnosed by ultrasonography, computed tomography or magnetic resonance imaging, and 4) excluding reasons, such as idiopathic thrombocytopenic purpura or hypersplenism. Read More

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December 2018
16 Reads

Hepatic Angiosarcoma with Kasabach-Merritt Phenomenon: A Case Report and Review of the Literature.

Ann Hepatol 2018 July - August ,;17(4):655-660

Department of Internal Medicine, Division of Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan.

A 76-year-old woman was referred to our hospital due to massive gingival bleeding following teeth extraction. Laboratory findings suggested disseminated intravascular coagulopathy (DIC). Enhanced computed tomography and magnetic resonance imaging disclosed multiple hypervascular liver masses of 2-6 cm in diameter, the largest of which displaying an irregular enhancement pattern. Read More

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June 2018
10 Reads

Impact of sirolimus treatment for refractory kaposiform hemangioendothelioma with exacerbation of the disease 10 years after initial diagnosis.

Rare Tumors 2018 30;10:2036361318776185. Epub 2018 May 30.

Department of Pediatrics, Kinki University Faculty of Medicine, Osaka-Sayama, Japan.

We describe our experience with a 12 year-old girl with kaposiform hemangioendothelioma accompanied by Kasabach-Merritt phenomenon with exacerbation of the disease 10 years after the initial diagnosis. Kaposiform hemangioendothelioma infiltrated into the subcutaneous tissue of the facial skin with deterioration of coagulopathy despite conventional therapies including corticosteroid, vincristine, and propranolol. Sirolimus, a mammalian target of rapamycin inhibitor, produced rapid and dramatic improvement of the Kasabach-Merritt phenomenon and kaposiform hemangioendothelioma shrinkage. Read More

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May 2018
1 Read

A case of an elderly female with diffuse hepatic hemangiomatosis complicated with multiple organic dysfunction and Kasabach-Merritt syndrome.

Clin J Gastroenterol 2018 Oct 29;11(5):411-416. 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

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October 2018
5 Reads

Atypically presenting kaposiform hemangioendothelioma of the knee: ultrasound findings.

J Med Ultrason (2001) 2018 Oct 10;45(4):653-656. Epub 2018 Apr 10.

Department of Radiology, Loyola University Medical Center, Maywood, IL, USA.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of early childhood and infancy. Kasabach-Merritt phenomenon, a common complication of KHE, is characterized by life-threatening thrombocytopenia, hemolytic anemia, and consumption coagulopathy. There may be atypical cases that do not present with Kasabach-Merritt phenomenon and do have atypical imaging findings. Read More

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October 2018
1 Read

[Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon].

An Pediatr (Barc) 2018 May 4;88(5):292-293. Epub 2018 Apr 4.

Servicio de Pediatría, Hospital Dr. José Molina Orosa, Lanzarote, Las Palmas, España.

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May 2018
3 Reads

A Case Report of 2 Sirolimus-Related Deaths Among Infants With Kaposiform Hemangioendotheliomas.

Pediatrics 2018 Apr;141(Suppl 5):S425-S429

Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital and School of Medicine, Shanghai Jiao Tong University, Shanghai, China

Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that is potentially life-threatening when presenting with Kasabach-Merritt phenomenon (KMP). KMP is clinically characterized as severe thrombocytopenia and hypofibrinogenemia and therefore is associated with a high mortality rate. There is no standard of cure for KHE currently. Read More

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April 2018
8 Reads

Pneumonia During Sirolimus Therapy for Kaposiform Hemangioendothelioma.

Pediatrics 2018 Apr;141(Suppl 5):S421-S424

Departments of Pediatrics and

Sirolimus is an effective therapy for children with kaposiform hemangioendothelioma with or without the Kasabach-Merritt phenomenon. We report the case of a child with kaposiform hemangioendothelioma and the Kasabach-Merritt phenomenon who developed pneumonia (PCP) while on sirolimus and a prednisolone taper, after lack of adequate response to prednisolone, propranolol, and vincristine. He had a prompt positive clinical and laboratory response to sirolimus, but 4 weeks after starting it, at the age of 4 months, he developed PCP. Read More

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April 2018
2 Reads

Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon.

Br J Dermatol 2018 Aug 24;179(2):457-463. Epub 2018 Apr 24.

Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, U.S.A.

Background: Few studies have reported the clinical features, complications and predictors of Kasabach-Merritt phenomenon (KMP) associated with Kaposiform haemangioendothelioma (KHE).

Objectives: To determine the clinical characteristics present at diagnosis and to identify features that may aid clinicians in managing KHE.

Methods: We conducted a cohort study of 146 patients diagnosed with KHE. Read More

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August 2018
173 Reads

[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

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June 2018
3 Reads

Vascular Tumors in Infants: Case Report and Review of Clinical, Histopathologic, and Immunohistochemical Characteristics of Infantile Hemangioma, Pyogenic Granuloma, Noninvoluting Congenital Hemangioma, Tufted Angioma, and Kaposiform Hemangioendothelioma.

Am J Dermatopathol 2018 Apr;40(4):231-239

Professor, Department of Dermatology, Mayo Clinic, Rochester, MN.

Vascular tumors in infants present a diagnostic and treatment dilemma for both clinicians and pathologists. Infantile hemangioma, the most common vascular tumor in infants, can be confused for other less common vascular tumors in infants. Correct and timely diagnosis is important, as some vascular tumors can be associated with life-threatening coagulopathy. Read More

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April 2018
13 Reads

Metastatic Choriocarcinoma Masquerading as a Congenital Glabellar Hemangioma.

Pediatr Dev Pathol 2018 03 16:1093526618765039. Epub 2018 Mar 16.

1 Department of Pathology and Laboratory Medicine, 14524 Phoenix Children's Hospital , Phoenix, Arizona.

Infantile choriocarcinoma (ICC) is a rare, highly malignant form of gestational trophoblastic neoplasia. Rapid diagnosis and initiation of treatment are paramount in reaching a successful outcome. Patients with these tumors typically present with a triad of anemia, hepatomegaly, and precocious puberty. Read More

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March 2018
7 Reads

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

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June 2018
3 Reads

Kasabach-Merritt Syndrome in an Adult

Turk J Haematol 2018 08 2;35(3):204-205. Epub 2018 Feb 2.

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Department of General Medicine, Pondicherry, India

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August 2018
1 Read

Sirolimus for treatment of Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon: a retrospective cohort study.

Br J Dermatol 2018 May 25;178(5):1213-1214. Epub 2018 Mar 25.

Department of Dermatology and Venereology, Capital Institute of Pediatrics, No. 2 YaBao Street, ChaoYang District, Beijing, 100020, China.

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May 2018
5 Reads

Successful management of steroid-resistant vascular tumors associated with the Kasabach-Merritt phenomenon using sirolimus.

J Dermatol 2018 May 21;45(5):580-583. Epub 2018 Jan 21.

Guangzhou Women and Children's Medical Center, Guangzhou Medical University, China.

Vascular tumors associated with Kasabach-Merritt phenomenon (KMP) are life-threatening and the mortality is as high as 10-30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid-resistant cases. Read More

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May 2018
7 Reads

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

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December 2017
7 Reads

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

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January 2018
7 Reads

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

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May 2018
30 Reads

[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

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March 2018
4 Reads

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

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October 2017
5 Reads

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

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October 2017
5 Reads

Successful Treatment of Cardiac Angiosarcoma Associated with Disseminated Intravascular Coagulation with Nab-Paclitaxel: A Case Report and Review of the Literature.

Case Rep Oncol 2017 Sep-Dec;10(3):863-870. Epub 2017 Sep 21.

Department of Clinical Oncology, Aichi Cancer Center Hospital, Nagoya, Japan.

Angiosarcoma of the heart is an uncommon soft tissue sarcoma. A few cases of disseminated intravascular coagulation (DIC) associated with angiosarcoma occurring in various organs, but not the heart, have been reported. Although taxane is commonly used in the treatment of metastatic angiosarcoma, data on the efficacy of nab-paclitaxel for angiosarcoma are limited. Read More

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September 2017
14 Reads

Episode of Kasabach-Merritt phenomenon following Japanese encephalitis vaccination: Case report.

Vaccine 2017 12 16;35(48 Pt B):6594-6597. Epub 2017 Oct 16.

Department of Cardiac Surgery, The Children's Hospital of Zhejiang University School of Medicine, Zhejiang, China. Electronic address:

Kasabach-Merritt phenomenon (KMP) is a rare potentially life-threatening consumptive coagulopathy characterized by thrombocytopenia and hypofibrinogenemia occurring associated with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). A 10-month old male infant, diagnosed with KHE on his left leg, underwent a rapid increase of the lesion and severe thrombocytopenia, one day after the first dose of inactivated Japanese encephalitis (JE) vaccination. The episode of KMP was treated successfully by steroid. Read More

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December 2017
4 Reads

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

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September 2017
12 Reads

[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

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September 2017
13 Reads

[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

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October 2017
4 Reads

The effects of sirolimus on Kasabach-Merritt phenomenon coagulopathy.

Br J Dermatol 2018 Feb 21;178(2):e114-e116. Epub 2017 Dec 21.

Department of Dermatology and Reference Centre for Genodermatoses and Rare Skin Diseases (MAGEC), Université Paris Descartes - Sorbonne Paris Cité, INSERM U1163, Institut Imagine, Paris, France.

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February 2018
2 Reads

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

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December 2017
5 Reads

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

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August 2017
9 Reads

Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus.

European J Pediatr Surg Rep 2017 Jan 28;5(1):e32-e35. Epub 2017 Jul 28.

Division of Vascular Anomalies, Department of Pediatric Surgery, La Paz Children's Hospital, Madrid, Spain.

 Kaposiform hemangioendothelioma (KHE) is a vascular tumor frequently associated with Kasabach-Merritt phenomenon (KMP), characterized by severe thrombocytopenia and consumptive coagulopathy. Visceral involvement in KHE is rare. In our recent experience, sirolimus has shown to be an effective treatment in cutaneous KHE, becoming indeed the treatment of choice in KMP. Read More

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January 2017
12 Reads

Kaposiform haemangioendothelioma in a nine-year-old boy with Kasabach-Merritt phenomenon.

Br J Haematol 2017 10 17;179(1). Epub 2017 Jul 17.

Department of Dermatology and Venereology, Capital Institute of Paediatrics, Beijing, China.

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October 2017
7 Reads

A case of kaposiform haemangioendothelioma successfully and safely treated with sirolimus.

Clin Exp Dermatol 2017 Oct 13;42(7):825-827. Epub 2017 Jul 13.

Department of Paediatric Dermatology, Bristol Royal Hospital for Children, Bristol, UK.

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October 2017
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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

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June 2017
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