Bleeding disorders and reduced bone density

In recent decays, quality of life and life expectancy of individuals with severe haemophilia A and B have been improved by better quality in treatment and more availability of blood coagulation products. Recently, new concerns have emerged in current groups such as auto-antibody inhibitors to coagulation factors and reduced bone density (RBD). RBD in bleeding disorders is becoming an escalating burden worldwide due to an increased in life expectancy and also in the ageing population with bleeding disorders. Here, we review published papers on bone mineral density of individuals with haemophilia A & B, a rare bleeding disorder entitled combined factor V and VIII deficiency and other rare bleeding disorders. It seems in individuals with bleeding disorders preventive measures including early detection, treatment and adequate physical activity, encouragement to be active in sports and finally anti-osteoporotic therapy must be done.     

High levels of Von Willebrand factor markers in COVID-19: a systematic review and meta-analysis

Clinical and Experimental Medicine - The SARS-CoV-2 virus has spread to all corners of the world. Thrombosis is the cause of organ failure and subsequent death in COVID-19. The pathophysiology of...     

Are individuals with severe haemophilia A prone to reduced bone density?

Individuals with severe haemophilia A may be at risk for reduced bone mineral density because of reduced weight-bearing exercise and hepatitis C infection. For confirming the reduced bone density, in the current cross-sectional study, we tried to address bone mineral density in individuals with severe haemophilia A and surveyed the relation of reduced bone density with hepatitis C viruses. To fulfil these aims, bone density and biochemical indexes in 18 individuals with severe haemophilia A and also in 18 individuals matched for age, sex, weight and height, as the control group, were examined. The obtained results showed that individuals with severe haemophilia A had reduced bone density (1.136 +/- 0.118, 0.801 +/- 0.238) in lumbar and femur regions, respectively, in comparison with the age- and sex-matched group (1.299 +/- 0.237, 1.458 +/- 0.505). The major complications of coagulation disorders are various types of excessive bleeding. The current study describes an association between severe haemophilia A and osteopenia, specifically at both the femur and the lumbar spine.     

Successful Use of Factor VIII Concentrate and Fresh Frozen Plasma for Four Dental Extractions in an Individual with Combined Factor V and VIII Deficiency

Zusammenfassung

Hintergrund

Die kombinierte Faktor-V/Faktor-VIII-Defizienz (CF5F8D) ist eine seltene autosomal rezessive Erbkrankheit mit einer Prävalenz von ca. 1:100 000 in der jüdischen Bev ö lkerung. Die betroffenen Personen haben nur 5–30% der normalen Faktor-V- und Faktor-VIII-Plasmakonzentrationen; andere Plasmaproteine zeigen normale Werte. Abhängig von der Schwere der Hämorrhagie wird diese Blutungsst ö rung mithilfe von Plasmainfusionen und Faktor-VIII-Konzentraten zur Kompensation des Faktor-V- und Faktor-VIII-Mangels behandelt.

Fallbericht

Ein 22 Jahre alter Mann mit CF5F8D, bei dem vier kari ö se Zähne (die ersten maxillären Prämolaren sowie der erste und zweite mandibuläre Molar) extrahiert werden mussten, wurde in unsere Klinik eingeliefert. Seine Blutwerte waren wie folgt: aPTT 51 s (normal 33 s), PT 27 s (normal 12 s), Faktor V 13% und Faktor VIII 21% der Normalwerte. Mit der Infusion von Faktor-VIII-Konzentrat wurde 12 h vor der ersten Zahnextraktion begonnen. Die Infusion wurde in 8-Stunden-Intervallen fortgesetzt. Darüber hinaus wurden 10 ml/kg frischgefrorenes Plasma zur Kompensation der Faktor-V-Defizienz appliziert. Während und nach den Eingriffen kam es zu keinen nennenswerten Blutungen. Die Plasmakonzentrationen für Faktor V bzw. VIII nach den Zahnextraktionen waren 64 bzw. 75%.

Schlussfolgerung

Plasmakonzentrationen für Faktor V und VIII von 60–75% der Normalwerte scheinen ausreichend zu sein, um gr ö βere kieferchirurgische Eingriffe vornehmen zu k ö nnen.     

Inherited factor V deficient neonate with galactosaemia

ObjectivesReporting a case of inherited factor V deficiency and galactosemia.MethodsA neonate was admitted with hematoma, jaundice, splenomegaly, diarrhea, anemia, abdominal ascites and bilateral cataracts that diagnosis of galactosaemia and factor V deficiency was established.ResultsCoinheritance of both coagulation disorder and metabolic disorder is very rare episode that was identified in a neonate.ConclusionOur case indicates that in mild bleeding episodes of neonates that imitate of coagulation disorders should be considered promptly by pediatricians.     

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus: review of literature

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE), is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII (fVIII) inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced fVIII activity and a high titre of fVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, fVIII inhibitor assay was negative.     

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE) is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced factor VIII activity and a high titer of FVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, factor VIII inhibitor assay was negative.