SummaryThe abnormal fibrinogen Haifa is characterized by the fact that calcium present during enzymatic digestion by plasmin does not protect the Haifa D gamma chain against further plasmin attack as it does in normal molecules.Since calcium binding to fibrinogen, ADP - platelet aggregation cofactor activity and gamma dimerization process induced by factor XIIIa are normal for fibrinogen Haifa, the corresponding sequences in the gamma chain are not involved. It seems rather that the anomaly resides near the gamma 302 plasmin cleavage site that is protected when calcium is bound to the gamma chain and that this affects the availability of the polymerization site located in the C terminal part of the chain.

Venous thrombosis, including superior and inferiorvena caval occlusion is considered an integral manifestation of Behçet’s disease. In this series, 5 of 11 patients (45.4%) with active Behçet’s disease had clinical evidence of present or past venous thrombosis. The mean euglobulin lysis time in the 11 subjects was found to be within the normal range: however, in 3 patients, significant impairment of fibrinolytic activity was demonstrated. Inhibition of systemic fibrinolysis may be a contributory factor in the pathogenesis of the thrombotic diathesis characteristic of Behçet’s disease.

A 30-year-old female presenting with arterial occlusions of the lower limbs was discovered to have a markedly abnormal thrombin time and reptilase time. Further investigations revealed the presence of a qualitatively abnormal fibrinogen. Two other family members who were completely asymptomatic had similar defects. The abnormal fibrinogen has been characterized and found to be a new variant and as such designated fibrinogen ‘Haifa’.

BackgroundPERSIST was a prospective, non‐interventional, real‐world study of guselkumab and ustekinumab in adult patients with moderate‐to‐severe plaque psoriasis in Germany.ObjectivesTo examine effectiveness, safety and quality‐of‐life (QoL) outcomes to Week (W) 104 of treatment with guselkumab and ustekinumab in patients with moderate‐to‐severe plaque psoriasis.MethodsPatients (≥18 years of age) received guselkumab or ustekinumab as per routine clinical practice. Outcomes to W104 were examined separately in guselkumab and ustekinumab recipients. An ad hoc exploratory analysis of outcomes with guselkumab versus ustekinumab was also performed following propensity score matching.ResultsOverall, 302 and 313 patients received guselkumab and ustekinumab, respectively. Patients in both cohorts experienced improvements in disease activity and QoL that were maintained to W104, with 64.7% and 63.6% of guselkumab‐ and 54.6% and 64.4% of ustekinumab‐treated patients achieving a Psoriasis Area and Severity Index (PASI) 90 response and a Dermatology Life Quality Index (DLQI) 0/1 score, respectively. Propensity score matching yielded well‐balanced baseline characteristics except for prior biologic use, which was higher in guselkumab versus ustekinumab recipients (51.7% vs. 32.0%). Achievement of PASI ≤1 at W104 was more common in guselkumab versus ustekinumab recipients (58.7% vs. 49.7%). The W104 PASI90 response rate was 65.6% with guselkumab and 56.0% with ustekinumab; corresponding rates for PASI100 were 44.3% and 28.5%. In guselkumab recipients, response rates were higher in biologic‐naïve versus biologic‐experienced patients (PASI90, 77.1% vs. 53.4%; PASI100, 55.0% vs. 33.0%). A high level of response for QoL outcomes was observed for both treatments.ConclusionsUstekinumab and guselkumab led to improvements in physician‐assessed and patient‐reported outcomes that were sustained for up to 2 years, with no new safety signals identified. Following propensity score matching, greater improvements in PASI outcomes were observed with guselkumab versus ustekinumab. Improvements with guselkumab were highest in biologic‐naïve patients, highlighting the value of early treatment.