Mining of mortality-related findings in rare bleeding disorders:a retrospective study from two centers

Background@#Rare bleeding disorders include inherited coagulation disorders except for von Willebrand disease and hemophilia A and B. These disorders affect both men and women worldwide and mainly have an autosomal recessive pattern of inheritance. Given the paucity of cases of rare bleeding disorders, there are limited data regarding some topics among bleeding disorders. @*Methods@#This retrospective study from 2005‒2019 collected demographic data and the causes of death among cases with rare bleeding disorders from 2 provinces of Iran. @*Results@#Overall, 5 deaths were reported, including 3 cases with factor V deficiency, a case with factor XIII deficiency, and a case with combined factor V and factor VIII deficiencies. The main causes of death were bleeding in the central nervous system (2 cases; 1 with factor V deficiency and 1 with combined factor XIII deficiency). Post-partum hemorrhage was the cause of death in a woman with factor V deficiency while anaphylaxis shock was the cause of death in the case with combined factor V and factor VIII deficiencies. A woman with factor V deficiency died from an internal bleeding episode. @*Conclusion@#Gathering data on the causes of death in rare bleeding disorders through worldwide registries can be helpful for the management of this rare group of bleeding disorders.

Mining of mortality-related findings in rare bleeding disorders:a retrospective study from two centers

Background@#Rare bleeding disorders include inherited coagulation disorders except for von Willebrand disease and hemophilia A and B. These disorders affect both men and women worldwide and mainly have an autosomal recessive pattern of inheritance. Given the paucity of cases of rare bleeding disorders, there are limited data regarding some topics among bleeding disorders. @*Methods@#This retrospective study from 2005‒2019 collected demographic data and the causes of death among cases with rare bleeding disorders from 2 provinces of Iran. @*Results@#Overall, 5 deaths were reported, including 3 cases with factor V deficiency, a case with factor XIII deficiency, and a case with combined factor V and factor VIII deficiencies. The main causes of death were bleeding in the central nervous system (2 cases; 1 with factor V deficiency and 1 with combined factor XIII deficiency). Post-partum hemorrhage was the cause of death in a woman with factor V deficiency while anaphylaxis shock was the cause of death in the case with combined factor V and factor VIII deficiencies. A woman with factor V deficiency died from an internal bleeding episode. @*Conclusion@#Gathering data on the causes of death in rare bleeding disorders through worldwide registries can be helpful for the management of this rare group of bleeding disorders.

Bone Density Status in Bleeding Disorders: Where Are We and What Needs to Be Done?

Bleeding disorders, including hemophilia, can be seen in every ethnic population in the world. Among various bleeding disorders, reduced bone density has been addressed in hemophilia A. In recent years, there has been an increasing interest in addressing osteopenia and osteoporosis in hemophilia A. There is little or no study about the possible susceptibility of other individuals with bleeding disorders to reduced bone density. Questions have been raised about the role of blood coagulation factors in bone mineralization. This review provides new insight and ideas for further survey in the field of bleeding disorders and reduced bone density.

Factor V and VIII inhibitor in patients with combined factor v and VIII deficiency

Patients with coagulation factor[s] deficiency who use coagulation therapy are susceptible to forming inhibitors against coagulation factor[s]. In this survey we detected factor V and VTQ inhibitor in ten patients with combined deficiency of factors V and VIII from north east of Iran [Khorassan province]. It was revealed in our survey that eight patients had both factor V and factor VIII inhibitors and two patients had none. Because factor V and factor VIII share approximately 40% amino acid sequence homology in their A and C domains, it remains to be elucidated if it is one molecule that recognizes both factor V and VIII or whether there are two inhibitor molecules against common sites