No difference in quality of life between persons with severe haemophilia A and B.

Introduction: Good health-related quality of life (HRQoL) is an important goal in the treatment of persons with haemophilia B (PwHB). Studies focusing on this population are limited, however, and data are insufficient.

Aim: To assess the HRQoL in PwHB and to compare this to data on persons with haemophilia A (PwHA), as well as to evaluate the impact of joint health on HRQoL and to identify areas of insufficient care.

Monitoring standard and extended half-life products in hemophilia: Assay discrepancies for factor VIII and IX in pre- and postinfusion samples.

Background: Monitoring hemophilia treatment with extended half-life products is challenging for coagulation laboratories since factor assays may show substantial differences between results obtained with the one-stage assay (OSA) and the chromogenic substrate assay (CSA).

Objectives: The aim of this study was to evaluate and compare different factor assays and global coagulation methods.

Methods: Factor VIII (FVIII) and IX (FIX) activities and global assay parameters were analyzed in pre- and postinfusion samples (5 patients 2 samples/product/method).

A simplified flow cytometric method for detection of inherited platelet disorders-A comparison to the gold standard light transmission aggregometry.

Background: Flow cytometric platelet activation has emerged as an alternative diagnostic test for inherited platelet disorders. It is, however, labor intensive and few studies have directly compared the performance of flow cytometric platelet activation (PACT) to light transmission aggregometry (LTA). The aims of this study were 1/ to develop a simplified flow cytometric platelet activation assay using microtiter plates and 2/ to correlate the outcome to gold standard method LTA, and to clinical bleeding assessment tool scores (BAT score).

The current status of prophylactic replacement therapy in children and adults with haemophilia.

Initiating prophylactic treatment at an early age is considered to be the optimal form of therapy for a child with haemophilia A or B. The pioneering long term experiences of prophylactic treatment from Sweden and The Netherlands demonstrated the benefit of prophylaxis in retrospective and observational studies. Decades later, these benefits were confirmed in a randomized controlled study in USA.

Cerebral sinus venous thromboses in children with acute lymphoblastic leukaemia - a multicentre study from the Nordic Society of Paediatric Haematology and Oncology.

We present a prospective multicentre cohort of 20 children with acute lymphoblastic leukaemia (ALL) and cerebral sinus venous thrombosis (CSVT). The study covers a period of 5 years and comprises 1038 children treated according to the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL 2008 protocol. The cumulative incidence of CSVT was 2%.