726 results match your criteria Kasabach-Merritt Syndrome

Outcomes of surgery for giant hepatic hemangioma.

BMC Surg 2021 Apr 8;21(1):186. Epub 2021 Apr 8.

Hepatopancreatobiliary Surgery, Department of general surgery, The First Affiliated Hospital of Anhui Medical University, 120# Wanshui Road, Hefei, 230022, Anhui, China.

Background: The surgical indications for liver hemangioma remain unclear.

Methods: Data from 152 patients with hepatic hemangioma who underwent hepatectomy between 2004 and 2019 were retrospectively reviewed. We analyzed characteristics including tumor size, surgical parameters, and variables associated with Kasabach-Merritt syndrome and compared the outcomes of laparoscopic and open hepatectomy. Read More

View Article and Full-Text PDF

Kaposiform Hemangioendothelioma of the Oral Cavity: A Rare Tumor with an Unusual Location.

Head Neck Pathol 2021 Mar 13. Epub 2021 Mar 13.

Oral Diagnosis, Department of Dentistry, Federal University of Juiz de Fora, Governador Valadares, Brazil.

Kaposiform hemangioendothelioma is a rare neoplasm with intermediate malignant behavior, mainly affecting infants and children. Involvement head and neck is uncommon, and there are only four cases reported in the oral cavity and oropharynx. Microscopically, it is characterized by a vascular proliferation permeated by spindle-to-ovoid cells resembling Kaposi sarcoma. Read More

View Article and Full-Text PDF

Combined Transarterial Sclerotherapy and Embolization with Systemic Chemotherapy Followed by Everolimus in Kaposiform Hemangioendothelioma with Kasabach-Merritt Syndrome.

J Vasc Interv Radiol 2021 05 9;32(5):768-771. Epub 2021 Mar 9.

Reconstructive Microsurgery Department, Indonesia National Cancer Center - Dharmais Cancer Hospital, Jalan Let. Jend. S. Parman, Kav., Jakarta Barat, DKI Jakarta 11420, Indonesia.

View Article and Full-Text PDF

Kaposiform haemangioendothelioma: magnetic resonance imaging features in 64 cases.

BMC Pediatr 2021 03 3;21(1):107. Epub 2021 Mar 3.

Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, 610041, Chengdu, China.

Background: Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive disorder. The presenting and imaging features of KHE can overlap with other vascular anomalies and tumours. We aimed to analyse the imaging findings of KHE disorder and highlight features most suggestive of this diagnosis. Read More

View Article and Full-Text PDF

Comparison of efficacy and safety of corticosteroid and vincristine in treating kaposiform hemangioendothelioma and tufted angioma: A multicenter prospective randomized controlled clinical trial.

J Dermatol 2021 May 19;48(5):576-584. Epub 2021 Feb 19.

Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China.

Kaposiform haemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors that can cause life-threatening Kasabach-Merritt phenomenon. No evidence-based treatment strategies have yet been established, and its management is still a challenge. The purpose of this multicenter prospective randomized controlled study was to evaluate and compare the efficacy of corticosteroid and vincristine (VCR) in the treatment of KHE and TA. Read More

View Article and Full-Text PDF

Kaposiform haemangioendothelioma of duodenum in a neonate.

BMJ Case Rep 2021 Feb 19;14(2). Epub 2021 Feb 19.

Department of Gastroenterology, GB Pant Hospital, New Delhi, India

Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Read More

View Article and Full-Text PDF
February 2021

[A case of neonatal mesenteric Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon].

Zhonghua Er Ke Za Zhi 2021 Feb;59(2):139-141

Department of Neonatology, First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China.

View Article and Full-Text PDF
February 2021

[Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case].

Arch Argent Pediatr 2021 02;119(1):e65-e69

Sección Hepatología Infantil y Trasplante Hepático, Hospital Universitario Austral, Pilar, provincia de Buenos Aires, Argentina.

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. Read More

View Article and Full-Text PDF
February 2021

A case of neonate effectively treated with everolimus for giant hepatic hemangioma complicated with congenital duodenal atresia and Kasabach-Merritt syndrome.

J Neonatal Perinatal Med 2020 Dec 3. Epub 2020 Dec 3.

Department of Pediatric Surgery, Fujita Health University Hospital, Aichi, Japan.

Background: Disseminated intravascular coagulation (DIC) with Kasabach-Merrit syndrome from a large hepatic hemangioma is life-threatening. We report a case of giant hepatic hemangioma of the newborn with KMS.

Results: The patient was born at 37 gestational weeks and 2 days via cesarean section; weight at birth was 2952 g. Read More

View Article and Full-Text PDF
December 2020

Kasabach-Merritt Syndrome.

Am J Med 2020 12;133(12):e747

Internal Medicine, San Camillo Hospital, Rome, Italy. Electronic address:

View Article and Full-Text PDF
December 2020

Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management.

Orphanet J Rare Dis 2020 11 7;15(1):313. Epub 2020 Nov 7.

Department of Pediatric Surgery, Chengdu Women and Children's Central Hospital, Chengdu, 610031, China.

Objectives: There are no cohort studies of chronic lymphedema in patients with kaposiform hemangioendothelioma (KHE). We sought to characterize the incidence, clinical features, risk factors and management of chronic lymphedema in patients with KHE.

Methods: We conducted a multicenter retrospective analysis of patients who had a minimum of 3 years of follow-up after the onset of KHE and/or Kasabach-Merritt phenomenon (KMP). Read More

View Article and Full-Text PDF
November 2020


Wiad Lek 2020 ;73(8):1785-1789


Objective: Combination of kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt phenomenon (KMP) in newborn children is a life-threatening constellation. The purpose of the study is the choice of the diagnostic and treatment methods in these patients and evaluating the effectiveness of treatment using radiological methods of investigation. The study enrolled 6 newborn patients with KHE within a period 2013 - 2018. Read More

View Article and Full-Text PDF
October 2020

Kasabach-Merritt phenomenon with cellulitis in infant.

BMJ Case Rep 2020 Sep 15;13(9). Epub 2020 Sep 15.

Department of Dermatology and Venereology, Universitas Sumatera Utara Fakultas Kedokteran, Medan, Indonesia.

Kasabach-Merritt phenomenon (KMP) is consumptive coagulopathy in large vascular lesion. This condition is specific in kaposiform haemangioendothelioma (KHE) and tufted angioma, associated with high mortality rate. This condition often involves the limbs, trunk and retroperitoneum. Read More

View Article and Full-Text PDF
September 2020

Diffuse infiltrative hemangioma of pancreas accompanied by Kasabach-Merritt Syndrome: A case report.

Chin Med J (Engl) 2020 09;133(18):2263-2265

Department of Special Examination, The Second Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong 250001, China.

View Article and Full-Text PDF
September 2020

Initial dose recommendation for sirolimus in paediatric kaposiform haemangioendothelioma patients based on population pharmacokinetics and pharmacogenomics.

J Int Med Res 2020 Aug;48(8):300060520947627

Department of Pharmacy, Children's Hospital of Fudan University, Shanghai, China.

Objective: Sirolimus has been used to treat paediatric kaposiform haemangioendothelioma patients. However, there is considerable pharmacokinetic variability among individuals, and it is difficult to develop an initial dosing regimen. The goal of the present study is to recommend an initial sirolimus dose in paediatric kaposiform haemangioendothelioma patients based on population pharmacokinetics and pharmacogenomics. Read More

View Article and Full-Text PDF

Kaposiform Hemangioendothelioma in the Thoracic Spine: A Case Report and Review of the Literature.

Int J Spine Surg 2020 Jun 30;14(3):426-432. Epub 2020 Jun 30.

Royal National Orthopaedic Hospital, Stanmore, London, United Kingdom.

Background: Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of childhood. It has been most commonly reported in the trunk, retroperitoneum, and extremities, but cases involving the head and neck have been described. While thought to have limited metastatic potential, it is associated with Kasabach-Merritt syndrome (KMS), a consumptive coagulopathy and profound thrombocytopenia that is associated with significant mortality. Read More

View Article and Full-Text PDF

Kaposiform hemangioendothelioma with overlapping features of rapidly involuting congenital hemangioma and a delayed complication of necrotizing fasciitis.

Pediatr Dermatol 2020 Sep 16;37(5):902-906. Epub 2020 Jul 16.

Dermatology Division, CHU Sainte-Justine, Montreal, Canada.

We report the case of a male infant born at term with kaposiform hemangioendothelioma (KHE) of the right forearm and coagulopathy. Our case was unusual as it involuted leaving subcutaneous atrophy and prominent veins, which are more commonly observed in rapidly involuting congenital hemangioma. At 3 years of age, the child developed recurrent superficial thrombophlebitis localized to the area where the KHE had regressed. Read More

View Article and Full-Text PDF
September 2020

Successful management of a pregnant woman with Kasabach-Merritt syndrome and preeclampsia: A case report.

Medicine (Baltimore) 2020 Jul;99(28):e21198

Department of Obstetrics.

Introduction: Kasabach-Merritt Syndrome (KMS) is an extremely rare disease in adults, which lead to consumptive coagulopathy characterized by severe hypofibrinogenemia and thrombocytopenia. PATIENT CONCERNS:: a 25-year-old Chinese pregnant women complicated by preeclampsia and KMS presented with refractory postpartum hemorrhage and incision bleeding after cesarean section.

Diagnosis: The diagnosis of KMS was made based on clinical manifestation of Kaposiform Hemangioendothelioma, severe hypofibrinogenemia and thrombocytopenia. Read More

View Article and Full-Text PDF

Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels.

Pediatr Blood Cancer 2020 09 7;67(9):e28529. Epub 2020 Jul 7.

Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.

Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. Read More

View Article and Full-Text PDF
September 2020

Giant Liver Hemangiomas: A Plea for Early Surgical Referral and Resection.

Case Rep Surg 2020 16;2020:5923787. Epub 2020 Jun 16.

School of Medicine and Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, Virginia 23298, USA.

Hepatic hemangiomas are the most common benign hepatic tumor. Current guidelines recommend surveillance imaging and reserving surgical intervention for symptomatic patients with giant liver hemangiomas (>5 cm). We present the case of a patient with a rapidly enlarging giant hepatic hemangioma initially managed by surveillance. Read More

View Article and Full-Text PDF

Erratum: Kasabach-Merritt syndrome with large cutaneous vascular tumors.


J Indian Assoc Pediatr Surg 2020 May-Jun;25(3):192. Epub 2020 Apr 11.

[This corrects the article on p. 33 in vol. 17, PMID: 22279364. Read More

View Article and Full-Text PDF

Reactivation of Kasabach-Merritt phenomenon in a young adult with a congenital kaposiform hemangioendothelioma.

Pediatr Dermatol 2020 Sep 16;37(5):958-959. Epub 2020 Jun 16.

Department of Pediatric Surgery, University Hospital La Paz, Madrid, Spain.

We present the rare case of a young adult who developed late recurrence of Kasabach-Merritt phenomenon in a congenital kaposiform hemangioendothelioma that had been quiescent since infancy. We postulate that the extensive and infiltrative nature of our patient's tumor, combined with a recent history of direct microtrauma from acupuncture, contributed to the development of this late complication. Read More

View Article and Full-Text PDF
September 2020

Massive liver haemangioma causing Kasabach-Merritt syndrome in an adult.

Ann R Coll Surg Engl 2020 Nov 15;102(9):e1-e4. Epub 2020 Jun 15.

Mount Lebanon Hospital, Mount Lebanon, Lebanon.

Liver haemangiomas are common, but their size very rarely exceeds 40cm. Most people with liver haemangiomas are asymptomatic, and diagnosis is usually made incidentally during imaging for other complaints. When a liver haemangioma is symptomatic or produces complications, surgical intervention may be warranted. Read More

View Article and Full-Text PDF
November 2020

Tangled Up in Red Cells: A Large Liver Mass with Intravascular Hemolysis.

Dig Dis Sci 2020 07;65(7):1960-1963

Division of Gastroenterology and Hepatology, University of New Mexico School of Medicine, Albuquerque, NM, 87131, USA.

View Article and Full-Text PDF

Bone Marrow Langerhans Cell Histiocytosis in Association with Kasabach-Merritt Syndrome: The Difficulty of a Differential Diagnosis.

Case Rep Oncol 2020 Jan-Apr;13(1):408-413. Epub 2020 Apr 14.

Department of Internal Medicine II, Professor Doutor Fernando Fonseca Hospital, Amadora, Portugal.

Langerhans cell histiocytosis is a rare haematological disorder with variable clinical findings and a high mortality rate. On the other hand, Kasabach-Merritt syndrome is of rare onset at adult age, requiring the simultaneous presentation of vascular lesion, thrombocytopenia, and consumptive coagulopathy. We present the first reported case of both diseases in a single patient and highlight the difficulties of diagnostic. Read More

View Article and Full-Text PDF

Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy.

BMC Pediatr 2020 04 27;20(1):187. Epub 2020 Apr 27.

Department of Pediatric Surgery, The Sun Yat-Sen Memorial Hospital of Sun Yat-Sen University, No. 107 Yanjiang West Road, Guangzhou, 510120, People's Republic of China.

Background: Systemic drug therapy is generally recommended for infant huge vascular anomalies associated with thrombocytopenia and coagulopathy, but some patients are not suitable due to drug unresponsiveness or life threatening conditions before the drug works, who will need to receive surgical treatment. This study retrospectively analyzed the clinical features, imaging features, and surgical outcomes of these patients.

Methods: The clinical data of 4 infants with huge vascular anomalies (2 vein malformations (VMs) and 2 kaposiform hemangioendothelioma (KHE)) associated with thrombocytopenia and coagulopathy treated from June 2016 to December 2017 were retrospectively analyzed. Read More

View Article and Full-Text PDF

Diffuse bone and soft tissue angiomatosis with GNAQ mutation.

Pathol Int 2020 Jul 20;70(7):452-457. Epub 2020 Apr 20.

Department of Translational Research and of New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy.

We describe a unique case of skeletal and extraskeletal angiomatosis complicated by Kasabach-Merritt syndrome. The patient was a 3-year-old boy, who presented with involvement of both femurs and left tibia, as well as with soft tissue lesions of the left thigh. At birth, multiple hemangiomas of the soft tissues of the frontal and parietal scalp had been identified, together with a space-occupying lesion of the lung. Read More

View Article and Full-Text PDF

Tufted angioma with Kasabach-Merritt syndrome: Two cases successfully treated with everolimus.

Pediatr Blood Cancer 2020 06 10;67(6):e28291. Epub 2020 Apr 10.

Department of Dermatology, Mohammed VI University Hospital Center, Cadi Ayyad University, Marrakech, Morocco.

View Article and Full-Text PDF

Diffused hepatic angiosarcoma with Kasabach-Merritt syndrome-case report and literature review.

BMC Gastroenterol 2020 Mar 30;20(1):80. Epub 2020 Mar 30.

Department of hepatobiliary surgery, Beijing Chaoyang Hospital, Capital Medical University, 8 Gongti South Street, Chaoyang, 100021, District Beijing, China.

Background: Hepatic angiosarcoma is a rare malignant tumor featured by highly aggressive behavior and poor prognosis. There are few reports about diffused hepatic angiosarcoma with Kasabach-Merritt syndrome till now.

Case Presentation: A male patient with the chief complain of hepatic space-occupying lesion accompanied by disturbance of consciousness and jaundice. Read More

View Article and Full-Text PDF