National strategic advocacy to manage patients with inherited bleeding disorders in low and lower-middle income countries

Expert Rev Hematol. 2023 Dec 15:1-14. doi: 10.1080/17474086.2023.2293092. Online ahead of print.ABSTRACTINTRODUCTION: Inherited bleeding disorders (IBDs) including hemophilia, von Willebrand disease, platelet disorders, mucocutaneous bleeding disorders and coagulation factor deficiencies are rarely found and under-recognized in low and lower-middle-income countries. Some patients succumbed to serious bleeding without diagnosis and treatment during childhood.AREA COVERED: Diagnosis, management, and prevention should be integrated into the existing health care system. Although some countries have not implemented appropriate health care infrastructure, an initiative plan should be set up by cooperation of experienced experts and health care providers. Identification of patients with IBDs should be started in the antenatal setting to search for females at risk of carrier state. The investigations include bleeding assessment, mixing venous clotting time, coagulogram, coagulation factor assay and mutation detection. Genotypic analysis is helpful for confirming the definite diagnosis, carrier detection as well as prenatal diagnosis for females at risk of bearing an offspring with severe bleeding manifestations. Management involves replacement therapy ranging from blood component to virus-inactivated factor concentrate. Appropriate research is an essential backbone for improving patients' care.EXPERT OPINION: Effective national strategic advocacy to manage patients with IBDs requires...

Source: Expert Review of Hematology - December 15, 2023 Category: Hematology Authors: Ampaiwan Chuansumrit Theera Ruchutrakul Nongnuch Sirachainan Pimpun Kitpoka Monratta Panuwannakorn Panyu Panburana Monthon Suwannuraks Nantana Sri-Udomporn Chusak Kijkunasathian Suphaneewan Jaovisidha Chirawat Utamakul Rungrote Natesirinilkul Bunchoo Pong Source Type: research