Journal of Blood Disorders & Transfusion

Managing hemophilia in underprivileged population

5^th World Hematologists Congress

August 18-19, 2016 London, UK

Aleem Jan

Sher-e-Kashmir Institute of Medical Sciences, India

Posters & Accepted Abstracts: J Blood Disord Transfus

Abstract:

A disease of royals with references to second century rabbinical rulings exempting boys from circumcision if two previous brothers had died of bleeding after the procedure, an Arab Physician Khalaf Ibn Abbass in his book �??Kitab al Tasrif�?� or Methods in Medicine (936-1013 AD) documented an illness localized to males of a particular village where patients profusely bled after minor trauma. By 12th century, Hebrew physician Moses Maimonides recognized that these bleeding tendencies were transmitted through mother. The term Hemophilia, originated with a German Friedrich Hopff (1882) who coined the name �??haemorrhaphilia�?� which later got abbreviated to hemophilia. Though hemophilia is often associated with family of Nicholas 11, the Tsar of Russia and Alexandra, granddaughter of Queen Victoria, but the bitter reality is that it occurs in all ethnic and racial groups throughout world. Hemophilia became known as royal disease since Queen Victoria was a carrier and passed this carrier status to many of her progeny daughters since consanguineous marriages was the order of day. History bears testimony to fact that Tsars of Russia fell apart because of preoccupation with management of their Hemophiliac son Alexei Nikolavich. Rusputin perhaps the infamous monk goes down in history with his role in management of Hemophilia. A sex linked recessive bleeding disorder classified as Hemophilia A with incidence of 1 per 5000 male births, Hemophilia Bwith 1 in 30,000and estimated number of Hemophiliacs worldwide is 400,000. Classified as severe, moderate and mild depending on factor levels which respectively are <1, 1-5, 5-50 per pooled plasma. Although 80% of hemophiliacs live in developing world and out of 191 member states of W.H.O., 143 developing countries fall within Asia, Africa and South America, yet there is a significant lack of data as data collection is improper and perhaps does not represent true epidemiology. This is further compounded by interruptions in availability of factors and more often than not components in form of FFP, Cryoprecipitate are being relied upon. Despite available robust screening there is always risk of transmission of disease and in one of our recently concluded study we found 21.1% prevalence for HCV, 0.9% for HBV, 0.4% for HIV. Studies across globe report prevalence of HCV infection in Hemophiliacs from 7.5 to more than 50% and multi transfusions being single most risk factor. Inhibitors contribute to miseries as well. So do target joint arthropathies. In nutshell Comprehensive Hemophilia care centers, availability of recombinant factor concentrate with nucleic acid testing (NAT) of blood could change outlook of Hemophilia management in underprivileged populace.

Biography :

Email: aleem_maleem@yahoo.co.in