Hemophilias are clinically relevant rare diseases: hemophilia A results from the deficiency or dysfunction of coagulation FVIII, and hemophilia B of FIX. Coagulation factors are key medicines for management of hemophilia. So, adequate supply of blood components is a very important aspect for health systems functioning. This includes the provision of plasma derivatives medicines.

To calculate the availability of plasma derived FVIII and FIX in hemophilia context, we used data from Annual Global Survey (AGS) 2018 by The World Federation of Hemophilia (WFH), which provides detailed information about demographic, epidemiologic and clinical aspects on people with hemophilia in 125 countries. This way, the inclusion and exclusion criteria correspond to the countries that reported the information in the AGS 2018. To calculate the need for plasma derived FVIII and FIX in the hemophilia set, we followed the 2017 European consensus on hemophilia management with coagulation factor, ratified by the WFH, establishing “the minimum consumption of factors VIII and IX in any country should be 4 UI and 0.5 UI per capita of general population respectively ”per year.

In our paper, this ‘unmet need'parameter was designed and adopted by the authors. This approach simply assumes the distance between where countries are (based on plasmatic coagulation factors use as found in data source) and where countries should be (considering de score recommended by the European consensus). FVIII : Among the 22 sub-Saharan Africa countries reporting plasmatic FVIII use, 11 of them had 100% of unmet need. Mauritania had 98.5%, South Africa had 70.7% and, in contrast, Mauritius had 18%. Likewise, among the seven countries of the south Asia reporting plasmatic FVIII use, all of them had between 97.1% and 100% of unmet need. FIX : In the 21 sub-Saharan Africa countries reporting plasmatic FIX use, nine of them (Cote d'Ivoire, Eritrea, Ethiopia, Kenya, Madagascar, Malawi, Mali, Nigeria and Senegal) had 100% of unmet need, Mauritania had 94.6%, Sudan had 93.8%, South Africa had 66.6% and, in contrast, Mauritius had 26.5%. Likewise, among the seven countries of the south Asia reporting plasmatic FIX use, all of them had between 99.5% and 100% of unmet need, except India (88.2%).

Assuming European consensus, only in 11 countries we have ideal availability of plasmatic FIX (10.4%) of 105 countries and only in four countries we have ideal availability of plasmatic FVIII (3.6%) of 111 countries. However, a large discrepancy exists in the scale of unmet need along socioeconomic lines, for example, the following low-income countries are the same that unmet need of plasmatic FVIII and FIX. Thus, we can realize that there is a correlation between the blood supply available and a possible plasma fractionation to obtain plasmatic factors to attend people with hemophilia.

A more detailed understanding of a country's plasma derived FVIII and FIX needs will allow stakeholders such as ministries of health and non-governmental entities that focus on global health, to better predict the needed of these factors and plan for adequate availability of plasma factors. The amount of unmet plasma derived FVIII and FIX needs globally reinforces that the target of 4 UI and 0.5 UI per capita, respectively, is inadequate to fulfil plasma derived FVIII and FIX needs for the vast majority of countries.