Hämangiom-Thrombozytopenie-Syndrom

Diffused
hepatic angiosarcoma with Kasabach-Merritt syndrome-case report and literature review. Abdominal computed tomography showed enlarged liver, irregular liver contours, heterogeneous hepatic parenchyma with diffusely distributed multiple hypodense focal lesions
bmcgastroenterol.biomedcentral.com • CC-by-4.0
Obstructive
jaundice caused by a giant liver hemangioma with Kasabach-Merritt syndrome: a case report. Radiological findings. a Computed tomography (CT) scan of the abdomen showing a well-demarcated liver mass consistent with hemangioma in the anterior segment of the right hepatic lobe to segment IV. The tumor pressed a bile duct in the hepatic portal region, causing an intrahepatic bile duct to expand. b A CT scan performed 5 years prior to the study shows a low-density tumor with a diameter of 28 mm in the right lobe. c The size of the tumor was shown to be 13 cm in diameter in magnetic resonance imaging, with a clearly demarcated border and a heterogeneous signal on T2-weighted images. The tumor had a fluid-fluid level at the tumor margins. d A stricture of approximately 2 cm in length in the common bile duct was observed via endoscopic retrograde cholangiography and suspected to be a result of compression from the outside wall. This caused partial expansion of a right lobe bile duct and high expansion of the left lobe bile duct
surgicalcasereports.springeropen.com • CC-by-4.0
Propranolol
in the preoperative treatment of Kasabach–Merritt syndrome: a case report. Magnetic resonance imaging of soft tissue mass in the neck – T1-weighted sequence
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Propranolol
in the preoperative treatment of Kasabach–Merritt syndrome: a case report. Magnetic resonance imaging of soft tissue mass in the neck – the contrast enhancement on T2-weighted sequence
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Kasabach-Merritt syndrome, also known as hemangioma thrombocytopenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumor is responsible for thrombocytopenia, microangiopathic hemolytic anemia and consumptive coagulopathy.

Pathology

Vascular tumors may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe cases, there may be disseminated intravascular coagulation.

Etiology

Kasabach-Merritt syndrome is usually caused by kaposiform hemangioendothelioma or tufted angioma. Other vascular tumors may be responsible, such as infantile hemangioma.

Radiographic features

The radiographic features are specific to each vascular tumor. They can be found in multiple locations, more frequently subcutaneous, musculoskeletal, splenic and hepatic in location.

Treatment and prognosis

Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolization and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors) .

Complications

Repetitive hemarthrosis can also occur, leading to a hemophilia-like arthropathy . Other complications include congestive heart failure and gastrointestinal bleeding .

History and etymology

The syndrome is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two pediatricians who first described the condition in 1940.

Siehe auch:
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Assoziationen und Differentialdiagnosen zu Hämangiom-Thrombozytopenie-Syndrom:
Hämangiom
 
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kongenitale
vaskuläre Malformationen
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