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    569 results match your criteria Kasabach-Merritt Syndrome

    1 OF 12

    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 Apr 19. 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

    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

    Characteristics and operative treatment of extremely giant liver hemangioma >20 cm.
    Surgery 2017 Jan 23. 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

    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

    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

    [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

    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

    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

    Combined Transarterial Embolization and Propranolol as Effective Treatment for Hepatic Hemangioma with Kasabach-Merritt Syndrome and Heart Failure in a Neonate.
    Pediatr Neonatol 2017 Apr 11;58(2):191-193. Epub 2016 Jun 11.
    Department of Pediatrics, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan; Department of Medical Education and Bioethics, National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address:

    Kaposiform haemangioendothelioma of the head and neck.
    Crit Rev Oncol Hematol 2016 Aug 16;104:156-68. Epub 2016 Jun 16.
    Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge University Hospitals NHS Trust, Cambridge CB2 0QQ, UK.
    Background: Kaposiform haemangioendothelioma (KHE) is a tumor of intermediate malignant potential derived from vascular endothelial cells. Due to rarity of head neck KHE (HN-KHE) this comprehensive review aims to compile, analyze and present details to develop a consensus and augment available literature on HN-KHE.

    Materials And Methods: A comprehensive literature search was performed on PUBMED/MEDLINE, EMBASE, CINAHL and Science Citation Index for HN-KHE using MeSH words. Read More

    Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon.
    J Pediatr Surg 2016 Jun 9;51(6):1047-50. Epub 2016 Apr 9.
    Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, FL. Electronic address:
    Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Read More

    Bullous Kaposiform Hemangioendothelioma Masquerading as Aplasia Cutis Congenita.
    Pediatr Dermatol 2016 Sep 24;33(5):e267-9. Epub 2016 Jun 24.
    Geisel School of Medicine, Dartmouth College, Hanover, New Hampshire.
    We present the case of a male infant with violaceous bullae on the scalp that were initially thought to be bullous aplasia cutis but at 3 months of age were diagnosed as a kaposiform hemangioendothelioma. This diagnosis should be considered when evaluating newborns with bullous plaques on the scalp that do not heal in the first 2-3 weeks of life. Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents as a violaceous to purpuric plaque at birth or early infancy. Read More

    Neck Kaposiform haemangioendothelioma in a Fischer's lovebird (Agapornis fischeri).
    Res Vet Sci 2016 Jun 31;106:112-5. Epub 2016 Mar 31.
    Immunology and Molecular Oncology, Veneto Institute of Oncology, IOV IRCCS, Padova, Italy.
    A six-year-old female Fischer's lovebird (Agapornis fischeri) presented at necropsy with a cutaneous mass on the neck, 3.5cm in diameter, yielding and with blood content. Histopathological findings showed a neoplasm characterized by proliferation of vascular endothelial cells. Read More

    Clinical Outcomes for Systemic Corticosteroids Versus Vincristine in Treating Kaposiform Hemangioendothelioma and Tufted Angioma.
    Medicine (Baltimore) 2016 May;95(20):e3431
    From the Department of Oral Surgery (XL, CY, SZ); and Department of Oral-Maxillofacial Head and Neck Surgery (JZ, LZ), Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai Key Laboratory of Stomatology, Shanghai, China; Key Laboratory of Orthopedic Implant (XQ), Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine; Jining Medical University (JL), Jining, Shandong Province; Pudong Institute of Preventive Medicine (WY), Fudan University, Shanghai, China.
    A meta-analysis was performed to evaluate the efficacy and safety of systemic corticosteroids versus those of vincristine in the treatment of kaposiform hemangioendothelioma (KHE) and tufted angioma (TA).A literature search of PubMed, Embase, and Web of Science was performed for clinical studies on systemic corticosteroid versus vincristine therapies in treating KHE/TA. Pooled relative risks (RRs) and response rate with 95% confidence intervals (CIs) were used to measure outcomes. Read More

    Hepatic Hemangioma with Kasabach-Merritt Syndrome in an Adult Patient.
    Korean J Gastroenterol 2016 Apr;67(4):220-223
    Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.
    Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. Read More

    Successful Propranolol Treatment of a Kaposiform Hemangioendothelioma Apparently Resistant to Propranolol.
    Pediatr Blood Cancer 2016 07 21;63(7):1290-2. Epub 2016 Apr 21.
    Neonatal Intensive Care Unit, Medical Surgical Fetal-Neonatal Department, "A. Meyer" University Children's Hospital, Florence, Italy.
    A newborn with unresectable kaposiform hemangioendothelioma associated with Kasabach Merritt phenomenon, unresponsive to vincristine and prednisone, received second-line treatment with propranolol at a dose of 2 mg/kg/day, starting at 2 months of life and continued for 13 months. There was only slight reduction in tumor mass, but measurement of propranolol levels showed extremely low plasma concentrations. The propranolol dose was progressively increased to 3. Read More

    Hepatic small vessel neoplasm, a rare infiltrative vascular neoplasm of uncertain malignant potential.
    Hum Pathol 2016 Aug 14;54:143-51. Epub 2016 Apr 14.
    University of California, San Francisco, Department of Pathology, San Francisco, California, United States, 94143.
    Characteristic but rare vascular neoplasms in the adult liver composed of small vessels with an infiltrative border were collected from an international group of collaborators over a 5-year period (N=17). These tumors were termed hepatic small vessel neoplasm (HSVN), and the histologic differential diagnosis was angiosarcoma (AS). The average age of patients was 54years (range, 24-83years). Read More

    Metastatic Angiosarcoma with Kasabach-Merritt Syndrome Responsive to Gemcitabine and Vinorelbine after Failure of Liposomal Doxorubicin and Paclitaxel: A Case Report.
    Case Rep Oncol 2016 Jan-Apr;9(1):177-81. Epub 2016 Mar 12.
    Winship Cancer Institute, Emory University, Atlanta, Ga., USA.
    Kasabach-Merritt syndrome (KMS) describes a consumptive coagulopathy associated with certain vascular tumors. It is thought that platelets are destroyed as they circulate through the aberrant endothelial surfaces associated with these tumors. Most published literature describes infants with kaposiform hemangioendothelioma, but a similar syndrome can complicate angiosarcoma in adults. Read More

    Recurrent Primary Angiosarcoma of the Breast Presenting as Kasabach-Merritt Syndrome: A Case Report and Review of Literature.
    J Clin Diagn Res 2016 Feb 1;10(2):XD04-XD07. Epub 2016 Feb 1.
    Professor and Head, Department of General Surgery, Bangalore Medical College and Research Institute , Bangalore, Karnataka, India .
    Angiosarcoma of the breast is an unusual tumour. The diagnosis of this condition is difficult clinically, pathologically and radiologically. Treatment includes surgery followed by chemoradiation. Read More

    [Clinicopathologic study of pediatric vascular anomalies: a report of 117 cases].
    Zhonghua Bing Li Xue Za Zhi 2016 Apr;45(4):252-7
    Department of Pathology, Guangzhou Women and Children's Medical Center, Guangzhou 510623, China.
    Objective: To study the clinicopathologic features of pediatric vascular anomalies and application of ISSVA classification.

    Methods: The clinical features, histopathologic findings and immunohistochemical results were analyzed in 117 cases of pediatric vascular anomalies encountered during the period from May 2014 to May 2015.

    Results: A total of 117 cases of vascular anomalies were studied. Read More

    Rapamycin-associated lymphoedema in an infant with Kasabach-Merritt phenomenon.
    Br J Dermatol 2016 Apr 20;174(4):933-4. Epub 2016 Jan 20.
    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.

    Obstructive jaundice caused by a giant liver hemangioma with Kasabach-Merritt syndrome: a case report.
    Surg Case Rep 2015 Dec 6;1(1):93. Epub 2015 Oct 6.
    Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
    Hemangioma is the most common benign tumor of the liver. Liver hemangioma (LH) usually remains asymptomatic, but the most common symptoms associated with LH are abdominal pain and discomfort. LH is an uncommon cause of bile duct dilatation and obstructive jaundice. Read More

    Orthotopic liver transplant for multifocal lymphangioendotheliomatosis with thrombocytopenia.
    Pediatr Transplant 2016 May 25;20(3):456-9. Epub 2016 Feb 25.
    Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Los Angeles, University of Southern California, Los Angeles, CA, USA.
    An eight-yr-old female with a history of multifocal lymphangioendotheliomatosis and thrombocytopenia presented for MVT. The patient had multiple vascular lesions in the skin and stomach in infancy. Although her cutaneous lesions resolved with vincristine and methylprednisolone, her gastric lesions persisted. Read More

    Treatment of Corticosteroid-Resistant Vascular Tumors Associated with the Kasabach-Merritt Phenomenon in Infants: An Approach with Transcatheter Arterial Embolization Plus Vincristine Therapy.
    J Vasc Interv Radiol 2016 Apr 15;27(4):569-75. Epub 2016 Feb 15.
    Department of Pathology, Guangzhou Women and Children's Medical Center, Guangzhou, Guangdong 510623, China.
    Purpose: To investigate the effectiveness and application of transcatheter arterial embolization (TAE) plus systemic vincristine for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants.

    Materials And Methods: TAE was performed in 17 infants (average age, 4.3 mo ± 2. Read More

    Tufted angioma of the maxilla: a rare case with unique clinical presentation.
    Oral Surg Oral Med Oral Pathol Oral Radiol 2016 Sep 19;122(3):e93-8. Epub 2015 Dec 19.
    Department of Oral Medicine and Pathology, School of Dentistry, National and Kapodistrian University of Athens, Athens, Greece.
    Tufted angioma is an uncommon benign vascular tumor usually presenting in early childhood and affecting mainly the skin. It has been associated with Kasabach-Merritt syndrome, a severe coagulopathy of poor prognosis. There have been very few published cases of oral tufted angiomas, and maxillary bone involvement has not been hitherto reported. Read More

    Abdominal Kaposiform Hemangioendothelioma Associated With Lymphangiomatosis Involving Mesentery and Ileum: A Case Report of MRI, CT, and 18F-FDG PET/CT Findings.
    Medicine (Baltimore) 2016 Feb;95(6):e2806
    From the Department of Nuclear Medicine (AD, CZ); Department of Gastroenterology (LZ); Department of Pathology (YW); and Department of Hepatopanreatobiliary Surgery (TH), Changhai Hospital, Second Military Medical University, Shanghai, China.
    Kaposiform hemangioendothelioma (KH) is a rare vascular tumor of intermediate malignancy that occurs mainly in the childhood. Adult patients with KH are rare. Imaging findings of KH have rarely been reported before. Read More

    Role of Sirolimus in Advanced Kaposiform Hemangioendothelioma.
    Pediatr Dermatol 2016 Mar-Apr;33(2):e88-92. Epub 2016 Feb 11.
    Department of Dermatology, University of California, San Francisco, San Francisco, California.
    Kaposiform hemangioendothelioma (KHE) is an infiltrative vascular tumor that classically presents in infancy. Management typically focuses on treating Kasabach-Merritt phenomenon (KMP), a disorder of severe and at times life-threatening platelet trapping. However, the morbidity of KHE extends beyond KMP. Read More

    Clinicopathological features of Kaposiform hemangioendothelioma.
    Int J Clin Exp Pathol 2015 1;8(10):13711-8. Epub 2015 Oct 1.
    Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University 7 Weiwu Road, Zhengzhou 450003, China.
    Kaposiform hemangioendothelioma (KHE), an intermediate tumor of endothelial origin in childhood, is often associated with Kasabach-Merritt phenomenon (KMP). In this study, 22 cases of KHE were immunochemically studied for CD31, CD34, ERG, smooth muscle actin (SMA), D240, GLUT1 and Ki67. The patients (15 males and 7 females) ranged in age from 13 days to 7 years (median, 2 mo). Read More

    [Comprehensive therapy for infant vascular tumor associated with Kasabach-Merritt phenomenon].
    Zhonghua Zheng Xing Wai Ke Za Zhi 2015 Jul;31(4):263-8
    Objective: To summarize the management of infant vascular tumors with Kasabach-Merritt phenomenon (KMP) and to evaluate the effect of drug combined with sclerotherapy.

    Methods: From Feb. 2007 to Nov. Read More

    Klippel-Trenaunay-Weber Syndrome with Kasabach-Merritt Coagulopathy and Hydronephrosis.
    Indian Pediatr 2015 Nov;52(11):987-8
    Department of Neonatology, Fortis Hospital, Noida, Uttar Pradesh, India. Correspondence to: Dr Supriya Bisht, Department of Neonatology, Fortis Hospital, B-22, Sector 62, Noida, Uttar Pradesh, India.
    Background: Klippel-Trenaunay-Weber Syndrome is a rare syndrome, consisting of vascular malformation of blood and lymph vessels.

    Case Characteristics: A newborn female with respiratory distress from birth, and having vascular malformation involving left thigh.

    Observation: The neonate also had hydronephrosis and developed complication of Kasabach Merritt syndrome. Read More

    Prenatal diagnosis of Klippel-Trenaunay-Weber syndrome with Kasabach-Merritt syndrome in utero.
    J Med Ultrason (2001) 2015 Jan 3;42(1):109-12. Epub 2014 Jul 3.
    Department of Obstetrics and Gynecology, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
    Klippel-Trenaunay-Weber syndrome (KTWS), a congenital disease characterized by cutaneous hemangiomas, soft tissue and bone hypertrophy, and occasionally arteriovenous malformations, is extremely rare and its natural history in utero is unknown. We present a prenatally diagnosed case of KTWS complicated with Kasabach-Merritt syndrome in utero and fetal hydrops from acute anemia. The fetus was diagnosed with KTWS at 24 weeks of gestation based on the ultrasound findings of hemangiomas and unilateral hypertrophy of the lower extremity. Read More

    Surgical Dead End in a Renal Transplant Recipient Associated With a Rare Thrombohemorrhagic Syndrome.
    Transplant Proc 2015 Oct;47(8):2537-40
    Transplantation Unit, Athens University School of Medicine, Laiko General Hospital, Athens, Greece.
    Background: Primary breast angiosarcoma is an extremely rare malignancy. Association of this type of tumor with Kasabach-Merritt syndrome has only been reported in 3 cases in the past. To our knowledge, this is the first reported case of a solid-organ recipient. Read More

    A tufted angioma.
    Indian Dermatol Online J 2015 Jul-Aug;6(4):266-8
    Department of Dermatology, Comprehensive Dermatology Centre, Hyderabad, Andhra Pradesh, India.
    Tufted angioma (TA) is a rare, benign, cutaneous angiomatous proliferation. It is more common in children, usually presenting as red-purple painful plaques on the trunk. We describe here a TA observed at nine months of age, appearing initially over the retroauricular area, gradually extending to involve skin of neck and trunk by two years of age. Read More

    Current Management of Vascular Tumors in the Neonate.
    Curr Pediatr Rev 2015 ;11(3):226-32
    Vascular Anomalies Center, La Paz Children´s Hospital and Associate Professor of Pediatrics. Universidad Autonoma, Madrid. Spain.
    Neonatal vascular tumours present particular difficulties in their diagnosis and treatment. The special behaviour of neonatal hemangioma and the aggressive but benign nature of kaposiform hemangioendothelioma provide examples of a modern need for a conservative approach. Recent advances in their pharmacological management have dramatically changed the therapeutical scope of this group of tumors with surgery playing a minor role in their treatment. Read More

    Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon.
    Br J Haematol 2015 Oct 30;171(1):38-51. Epub 2015 Jun 30.
    Department of Haematology, Our Lady's Children's Hospital, Dublin, Ireland.
    Kasabach-Merritt Phenomenon (KMP) refers to the clinical constellation of thrombocytopenia, consumptive coagulopathy and purpura associated with Kaposiform haemangioedothelioma or tufted angioma, but not the more common infantile haemangioma. It shows a variable and unpredictable response to traditional pharmacological agents, such as steroids, vincristine or interferon alpha 2a or 2b. More recently, the interaction between platelets and endothelial cells and the proangiogenic phenotype that results has been recognized to underly the pathogenesis of this disorder. Read More

    [One case of nasal Kaposiform hemangioendothelioma].
    Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2015 Feb;29(4):373-5
    Female patients, 41 years old, with the left nasal cavity bleeding intermittently and left nasal congestion 20 days as the chief complaint to the hospital. Physical examinationindicated dark red mass was at the front-end of left nasal cavity, which has not smooth surface with blood vessels and hemorrhagic secretions, and back up to the middle turbinate. Sinus enhancement 3D-CT showed soft tissue density can be found in the left nasal cavity,Scan CT value is 37-47 HU, and enhanced and delay is about 69-78 HU. Read More

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