First case of very late-onset FHL2 in Spain with two variants in the PRF1 gene.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by the proliferation and infiltration of macrophages and hyperactivated T lymphocytes that escape from the physiological control pathways and favour the existence of an environment of excessive inflammation and tissue destruction. HLH has been classified into two types: a primary or familial autosomal recessive form, caused by mutations in genes encoding proteins involved in the granule-dependent cytotoxic pathway (familial hemophagocytic lymphohistiocytosis [FHL] types 1-5); and other secondary or acquired form, generally associated with infections, malignancy, autoimmune diseases, metabolic disorders or primary immunodeficiencies. Since the first familial hemophagocytic lymphohistiocytosis-2 (FHL2) causative mutation in the gene was described in 1999, more than 200 mutations have been identified to date.

Correction to: Long-term effectiveness and safety of self-management of oral anticoagulants in real-world settings.

After publication of the original article [1], we were notified that the name of the software mentioned in the Background section is TAONET and not Tao Net.

Long-term effectiveness and safety of self-management of oral anticoagulants in real-world settings.

Background: The patient self-management (PSM) is an effective approach for controlling the international normalized ratio, INR, within the therapeutic range. Nevertheless, most of the literature derives from randomized clinical trials, and no from routine clinical practice. The main objective of the present study was to evaluate long-term effectiveness and safety of PSM of oral anticoagulants (OACs) in real-world settings.

Self-monitoring of oral anticoagulation: systematic review and meta-analysis of individual patient data.

Background: Uptake of self-testing and self-management of oral anticoagulation [corrected] has remained inconsistent, despite good evidence of their effectiveness. To clarify the value of self-monitoring of oral anticoagulation, we did a meta-analysis of individual patient data addressing several important gaps in the evidence, including an estimate of the effect on time to death, first major haemorrhage, and thromboembolism.

Methods: We searched Ovid versions of Embase (1980-2009) and Medline (1966-2009), limiting searches to randomised trials with a maximally sensitive strategy.

Comparing self-management of oral anticoagulant therapy with clinic management: a randomized trial.

Background: Control of oral anticoagulant treatment has been reported to be suboptimal, but previous studies suggest that patient self-management improves control.

Objective: To compare the quality of control and the clinical outcomes of oral anticoagulant treatment in self-managed patients versus patients following conventional management.

Design: Randomized, controlled trial.