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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Hereditary hemorrhagic telangiectasia and disseminated intravascular coagulation: a new clinical syndrome.

In summary, 47 patients with documented HHT and a bleeding problem were referred to San Joaquin Hematology Oncology Medical Group over a 2-year period. Fifty-one percent of patients were noted to have an associated DIC syndrome and of these 24 patients, 19 had acute DIC episodes, six had chronic DIC, six presented with diffuse, recurrent deep venous thrombosis and of these six, three suffered pulmonary emboli. Other defects were also noted and were thought to be coincidental defects. This syndrome should be readily considered and searched for when seeing patients with HHT, especially if significant hemorrhage or thrombosis is present. It should further be appreciated that many patients with HHT and bleeding are candidates for the development of acute or chronic DIC and thus a "mini" Kasabach-Merritt syndrome. When patients with HHT present with undue bleeding, this syndrome should be appreciated, searched for from the clinical and laboratory standpoint and, when found, treated in the appropriate manner with supportive therapy, mini-heparin or antiplatelet therapy as the clinical situation dictates.[1]


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