785 results match your criteria Kasabach-Merritt Syndrome

A Case of Infantile Hepatic Hemangioendothelioma/Hemangioma at Maharaj Nakorn Chiang Mai Hospital.

Cureus 2022 May 23;14(5):e25240. Epub 2022 May 23.

Department of Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, THA.

Infantile hepatic hemangioendothelioma/hemangioma is the most common benign hepatic vascular tumor of infancy, comprising approximately 1% of all childhood tumors. The tumor can present during the fetal or neonatal period as a hepatic mass. Common presentations include abdominal distension and a palpable hepatic mass. Read More

View Article and Full-Text PDF

Lower Gastrointestinal Bleeding Because of Kasabach-Merritt Syndrome Showing an Impressive Response to Sirolimus.

ACG Case Rep J 2022 Jun 9;9(6):e00688. Epub 2022 Jun 9.

Division of Gastroenterology and Hepatology, Internal Medicine Department, University of Puerto Rico-Medical Sciences Campus, San Juan, Puerto Rico.

Kasabach-Merritt syndrome is a rare but life-threatening disease in which a rapidly growing vascular tumor induces localized intravascular coagulation, causing thrombocytopenia, microangiopathic hemolytic anemia, and consumption coagulopathy. It presents mainly in infants and young children. We present an adult with recurrent and severe lower gastrointestinal bleeding due to Kasabach-Merritt syndrome, treated successfully with sirolimus after multiple other failed interventions. Read More

View Article and Full-Text PDF

A Comprehensive Review of Hepatic Hemangioma Management.

J Gastrointest Surg 2022 Jun 15. Epub 2022 Jun 15.

Department of Surgery, The Ohio State University Wexner Medical Center, 395 W. 12th Ave., Suite 670, Columbus, OH, USA.

Background: Hepatic hemangiomas (HHs) are benign liver lesions often discovered incidentally on imaging for various unrelated pathologies. We herein review the etiology, classification, diagnostic imaging, and management of HHs.

Methods: A comprehensive systematic review was performed utilizing MEDLINE/PubMed and Web of Science databases, with the end of search date being March 1, 2022, regarding HH diagnosis, imaging, and management. Read More

View Article and Full-Text PDF

Spinal kaposiform hemangioendotheliomas: is there a role for endovascular treatment?

Pediatr Neurosurg 2022 Jun 13. Epub 2022 Jun 13.

Kaposiform hemangioendotheliomas (KHE) are rare vascular neoplasms composed of groups of capillaries and endothelial cells, forming aneurysms and arteriovenous fistulas. They occur mainly in the extremities during early childhood or infancy, and although they are locally aggressive, the occurrence of metastases is uncommon. A 9-month-old male infant born with a thoracolumbar cutaneous red lesion presented with progressive loss of strength in lower limbs, evolving with progressive flaccid paraplegia and areflexia. Read More

View Article and Full-Text PDF

A rare case of Kasabach Merritt Syndrome presenting with an infantile hemangioma: A case report.

Ann Med Surg (Lond) 2022 Apr 29;76:103557. Epub 2022 Mar 29.

Department of Pediatrics, Pediatrics Neurointervention, King Fahad Armed Forces Hospital, Jeddah, Saudi Arabia.

Introduction: and importance: Infantile hemangioma, being a benign tumor of the blood vessel, is part of a triad composed of also thrombocytopenia and hypofibrinogenemia as part of Kasabach Merrit Syndrome.

Case Presentation: We report the case of a 2 months old female Saudi infant referred due to respiratory distress, thrombocytopenia, and enlarging hemangioma on right upper chest, neck, and lower cheek. Diagnosis of kaposiform hemangioendothelioma complicated by Kasabach Merritt thrombocytopenia was done based on the clinical triad of thrombocytopenia, bleeding tendency, and the presence of a vascular tumor. Read More

View Article and Full-Text PDF

Rapamycin induces autophagy and apoptosis in Kaposiform hemangioendothelioma primary cells in vitro.

J Pediatr Surg 2022 Jul 13;57(7):1274-1280. Epub 2022 Mar 13.

Department of Pediatric Oncology, Children's Hospital of Fudan University, Shanghai, China. Electronic address:

Background: Rapamycin has been recommended to treat Kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP), but the underlying mechanism of the clinical effect has not been established. Therefore, we determined rapamycin cytotoxicity on KHE cells in vitro and the underlying mechanism.

Methods: KHE primary cells were derived from a tumor specimen and treated with rapamycin. Read More

View Article and Full-Text PDF

MRI-based radiomics in distinguishing Kaposiform hemangioendothelioma (KHE) and fibro-adipose vascular anomaly (FAVA) in extremities: A preliminary retrospective study.

J Pediatr Surg 2022 Jul 13;57(7):1228-1234. Epub 2022 Mar 13.

Department of Radiology, Children's Hospital of Fudan University, Shanghai 201102, China. Electronic address:

Objective: To investigate the pretreatment differentiation between Kaposiform hemangioendothelioma (KHE) and fibro-adipose vascular anomaly (FAVA) in extremities of pediatric patients. To build and validate an MRI-based radiomic model.

Method: In this retrospective study, we obtained imaging data from 43 patients. Read More

View Article and Full-Text PDF

Long-term outcomes of low-dose radiotherapy in Kasabach-Merritt syndrome.

Radiat Oncol J 2022 Mar 22;40(1):45-52. Epub 2022 Mar 22.

Department of Radiation Oncology, Seoul National University College of Medicine, Seoul, Korea.

Purpose: Reports on results of radiation therapy (RT) for Kasabach-Merritt syndrome (KMS) are limited. We performed a retrospective study to evaluate the response rates and late complications and to determine the adequate RT dose for patients with KMS patients.

Materials And Methods: We studied 11 patients who received RT between October 1988 and September 2008 for KMS refractory to pharmacologic therapy. Read More

View Article and Full-Text PDF

Targeting inflammation-induced Kasabach-Merritt phenomenon.

Blood 2022 03;139(11):1603-1605

University of Colorado School of Medicine.

View Article and Full-Text PDF

A case of sirolimus treatment of kaposiform hemangioendothelioma in the neck.

Pediatr Int 2022 Jan;64(1):e15000

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

View Article and Full-Text PDF
January 2022

Management of sirolimus treatment for tumours associated with Kasabach-Merritt phenomenon.

J Eur Acad Dermatol Venereol 2022 07 21;36(7):e586-e588. Epub 2022 Mar 21.

Department of Dermatology and Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), University of Paris, Paris-Centre, Institut Imagine, University Hospital Necker-Enfants Malades, APHP5, Paris, France.

View Article and Full-Text PDF

[Clinicopathological features of congenital hemangioma: a study of 40 cases].

Zhonghua Bing Li Xue Za Zhi 2022 Mar;51(3):202-206

Department of Ultrasonography, Henan Provincial People's Hospital, Zhengzhou 450003, China.

To investigate the clinical and pathologic features, diagnosis and differential diagnosis of congenital hemangioma (CH). Forty cases of CH were diagnosed from January 2017 to December 2020 in Henan Provincial People's Hospital. The clinical and pathological and immunohistochemical data were analyzed, with review of literature. Read More

View Article and Full-Text PDF

Fetal neck giant hemangioma associated with postnatal arising Kasabach-Merritt syndrome.

Ceska Gynekol 2022 ;87(1):43-46

We report a prenatal ultrasound dia-gnosis of giant neck hemangioma at 30+1 weeks in a fetus resulting in the postnatal development of Kasabach-Merritt syndrome. Ultrasound scan revealed a large isoechoic mass occupying the whole neck, infiltrating the nasopharyngeal cavity, tongue, lower lip and mandible. Complex sonographic visualization with 2D and 4D was helpful in the process of parental counseling. Read More

View Article and Full-Text PDF

Cellular variant of kaposiform lymphangiomatosis: a report of three cases, expanding the morphologic and molecular genetic spectrum of this rare entity.

Hum Pathol 2022 04 21;122:72-81. Epub 2022 Feb 21.

Mayo Clinic, Department Laboratory Medicine and Pathology, Rochester, MN, USA, 55905. Electronic address:

Kaposiform lymphangiomatosis (KLA) is a very rare form of generalized lymphatic anomaly, consisting of a diffuse proliferation of abnormal, dilated lymphatics, and small fascicles of hemosiderin-laden spindled lymphatic endothelial cells. KLA occurs in children and young adults and may present with multicentric disease, pleural and pericardial effusions, and life-threatening coagulopathy. Genetically, KLA most often harbors somatic activating mutations in NRAS. Read More

View Article and Full-Text PDF

Kasabach-Merritt syndrome: a rare cause of swelling thigh in a Cambodian newborn.

Oxf Med Case Reports 2022 Feb 19;2022(2):omac004. Epub 2022 Feb 19.

Neonatal Intensive Care Unit, Calmette Hospital, Phnom Penh, Cambodia.

View Article and Full-Text PDF
February 2022

Primary Kaposiform Hemangioendothelioma of the Humerus: A Case Report.

Cureus 2022 Jan 15;14(1):e21262. Epub 2022 Jan 15.

Medical School, Shifa Tameer-E-Millat University, Shifa College of Medicine, Islamabad, PAK.

Kaposiform hemangioendothelioma (KHE) is a neoplasm originating mainly from vessels and has a mild proclivity for malignancy. This neoplasm mainly involves somatic soft tissue and retroperitoneum. Histological findings include a nodular arrangement of oval-to-spindle cells containing pale cytoplasm. Read More

View Article and Full-Text PDF
January 2022

The Impact of COVID-19 On Comorbidities: A Review Of Recent Updates For Combating It.

Saudi J Biol Sci 2022 May 10;29(5):3586-3599. Epub 2022 Feb 10.

Department of Pharmacology and Toxicology, College of Pharmacy, University of Hail, Hail, Saudi Arabia.

Coronavirus disease is caused by the SARS-CoV-2 virus. The virus first appeared in Wuhan (China) in December 2019 and has spread globally. Till now, it affected 269 million people with 5. Read More

View Article and Full-Text PDF

Rapidly involuting congenital haemangioma: diagnostic and therapeutic approach regarding two case reports.

BMJ Case Rep 2022 Feb 9;15(2). Epub 2022 Feb 9.

Radiology Department, Dona Estefânia's Hospital, Lisbon Central Hospital Center EPE, Lisbon, Portugal.

Two unrelated neonates were born with a large purplish congenital mass of the thigh and forearm. Both showed signs of heart dysfunction, and one of them had anaemia and thrombocytopenia. The imaging assessment of the lesions showed well-defined subcutaneous solid masses with an exuberant vascular component. Read More

View Article and Full-Text PDF
February 2022

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

Indian J Pathol Microbiol 2022 Jan-Mar;65(1):167-169

Department of Pediatric Oncology, TMC, Kolkata, West Bengal, India.

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. Read More

View Article and Full-Text PDF
February 2022

Sirolimus plus prednisolone vs sirolimus monotherapy for kaposiform hemangioendothelioma: a randomized clinical trial.

Blood 2022 03;139(11):1619-1630

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

The Kasabach-Merritt phenomenon (KMP) in kaposiform hemangioendothelioma (KHE) is characterized by life-threatening thrombocytopenia and consumptive coagulopathy. This study compared the efficacy and safety of sirolimus plus prednisolone vs sirolimus monotherapy as treatment strategies for KHE with KMP in the largest cohort to date. Participants were randomized to receive either sirolimus in combination with a short course of prednisolone or sirolimus monotherapy for at least 12 months. Read More

View Article and Full-Text PDF

18F-FDG PET/CT Findings in a Woman With Kaposiform Hemangioendothelioma in the Sacrum.

Clin Nucl Med 2022 Apr;47(4):e353-e354

From the Department of Nuclear Medicine, Anqing Municipal Hospital of Anhui Province, Anqing, China.

Abstract: Kaposiform hemangioendothelioma is a rare vascular tumor with borderline malignancy and is typically diagnosed in infancy or early childhood. It most commonly affects cutaneous tissues, whereas the subtype with only primary bone involvement is extremely rare. Herein, we report a case of Kaposiform hemangioendothelioma involving the sacrum in a 37-year-old woman, with intense 18F-FDG accumulation in the lytic lesion on PET/CT. Read More

View Article and Full-Text PDF

Kaposiform hemangioendothelioma of leg: An unusual presentation.

Indian J Cancer 2021 Oct-Dec;58(4):631-634

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

View Article and Full-Text PDF
February 2022

Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.

Br J Clin Pharmacol 2022 06 18;88(6):2769-2781. Epub 2022 Jan 18.

Radboud University Medical centre (Radboudumc), Nijmegen, Gelderland, the Netherlands.

Aims: Management of kaposiform haemangioendotheliomas (KHE) with Kasabach-Merritt phenomenon is challenging in young infants who are subjected to developmental pharmacokinetic changes. Sirolimus, sometimes combined with corticosteroids, can be used as an effective treatment of KHE. Simultaneously, toxicities such as interstitial pneumonitis related to the use of sirolimus may be fatal. Read More

View Article and Full-Text PDF

Transarterial Embolization in Neonatal Kasabach-Merritt Syndrome.

Front Pediatr 2021 1;9:788120. Epub 2021 Dec 1.

Department of Neonatology, Anhui Provincial Children's Hospital, Anhui Medical University, Hefei, China.

Kasabach-Merritt syndrome (KMS) is characterized by large hemangiomas and persistent thrombocytopenia, which may result in visceral hemorrhage and disseminated intravascular coagulation. This study aimed to evaluate the value of transarterial embolization (TAE) in neonatal KMS patients. The clinical course of 11 neonates with KMS who underwent TAE in the Department of Neonatology, Anhui Provincal Children's Hospital, Anhui Medical University, China, were reviewed retrospectively. Read More

View Article and Full-Text PDF
December 2021

Cutaneous and giant hepatic haemangioma associated with Kasabach-Merritt syndrome in an adult patient with discoid lupus erythematosus.

Wei Gao Xihu Qin

Postepy Dermatol Alergol 2021 Oct 5;38(5):909-911. Epub 2021 Nov 5.

Derpartment of General Surgery, The Affiliated Changzhou No. 2 People's Hospital of Nanjing Medical University, Changzhou, Jiangsu, China.

View Article and Full-Text PDF
October 2021

Kaposiform hemangioendothelioma of the bone in children and adolescents.

Pediatr Blood Cancer 2022 01 4;69(1):e29392. Epub 2021 Oct 4.

Cancer and Blood Disease Institute, Division of Hematology-Oncology, Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California.

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that mainly occurs during infancy or early childhood. Approximately 70% of cases are complicated by Kasabach-Merritt phenomenon. Although osseous extension of the primary lesion is relatively common, primary bone involvement by KHE is rare. Read More

View Article and Full-Text PDF
January 2022

Surgical Management of Giant Hepatic Haemangioma - Need for Redefining the Nomenclature According to the Size.

Pol Przegl Chir 2021 Mar;93(4):28-34

Department of General Surgery, Post Graduate Institute of Medical Education & Research, Chandigarh, India.

Introduction: Haemangiomas are the most common benign tumours of the liver. Most of them are asymptomatic. Giant hepatic haemangioma is defined as size greater than 5 cm in diameter. Read More

View Article and Full-Text PDF