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Correspondence
Adult hepatic epitheloid haemangioendothelioma presenting with Kasabach–Merrit syndrome: a case report
  1. B Ozturk1,
  2. U Coskun1,
  3. E Yaman1,
  4. A Cakir2,
  5. U O Akdemir3,
  6. R Yıldız1,
  7. G Akyol2,
  8. U Demirci1,
  9. S Buyukberber1,
  10. E Tezel4,
  11. M Benekli1
  1. 1
    Department of Medical Oncology, Gazi University Medical School, Ankara, Turkey
  2. 2
    Department of Pathology, Gazi University Medical School, Ankara, Turkey
  3. 3
    Department of Nuclear Medicine, Gazi University Medical School, Ankara, Turkey
  4. 4
    Department of General Surgery, Gazi University Medical School, Ankara, Turkey
  1. Correspondence to Dr U Coskun, Department of Medical Oncology, Gazi University Medical School, Besevler, Ankara 06500, Turkey; ugurcos{at}hotmail.com

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Kasabach-Merrit syndrome (KMS) is a serious consumptive coagulopathy with thrombocytopenia, associated with benign or malignant vascular tumours or malformations. Although it is generally reported with childhood vascular tumours, there is limited data about adult vascular tumours associated with KMS. Hepatic epithelioid haemangioendothelioma (HEHE) is a rare vascular neoplasm of the liver with intermediate malignant potential. In adult patients, HEHE associated KMS has reported in several case reports. A 39-year-old male patient with epitheloid haemangioendothelioma in the liver and KMS is described here. He presented with multiple bilobar liver metastasis and severe thrombocytopenia, anaemia and coagulopathy. Immunohistochemistry showed diffuse cytoplasmic staining with CD34 and vimentin; thus the diagnosis of epitheloid haemangioendothelioma was confirmed. It is believed that this patient is the third case with adult HEHE associated with KMS reported in the literature.

Hepatic epitheloid haemangioendothelioma (HEHE) is a rare vascular neoplasm of the liver with low malignant potential.1 Kasabach–Merrit syndrome (KMS) is serious coagulopathy and is rarely associated …

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