Laboratory Methods in the Assessment of Hereditary Hemostatic Disorders.

Hematol Oncol Clin North Am

Coagulation and Transfusion Medicine, Rhode Island Hospital and Warren Alpert School of Medicine at Brown University, 593 Eddy Street, Providence, RI 02903, USA. Electronic address:

Published: December 2021

In patients presenting with a suspect hereditary bleeding disorder a detailed bleeding history is first obtained. Testing proceeds in a tiered manner with platelet count, platelet morphology, platelet histogram, PFA-100, fibrinogen, prothrombin time, and activated partial thromboplastin time. More detailed testing includes von Willebrand factor, individual clotting factor assays, and platelet function testing. Next, testing for a dysfibrinogenemia, FXIII, or a fibrinolytic defect is considered. Hemostatic abnormality is not demonstrated in a fraction of patients. An approach to management in these patients, such as desmopressin or antifibrinolytic therapy, may be required and empiric use of blood component therapy is discouraged.

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http://dx.doi.org/10.1016/j.hoc.2021.07.002DOI Listing

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