The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed-up for a median time of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only one patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy (IST). Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), while no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within two months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of non-survivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other non-survivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.

Patients with thrombotic antiphospholipid syndrome (APS) require long-term anticoagulation due to the high-thrombotic recurrence risk. Vitamin K antagonists (VKA) have been traditionally considered the standard of care in thrombotic APS. Nevertheless, the risk of recurrence persists with VKA. There are publications considering different intensities of anticoagulation with VKA; however, the standard-intensity anticoagulation (international normalized ratio between 2.0 and 3.0) is the most recommended. Furthermore, there is no consensus on the role of antiplatelet treatment in thrombotic APS. Nonvitamin K antagonist oral anticoagulants (NOACs) have emerged as an alternative to VKA for many indications. There are, however, discrepancies regarding the management with NOACs in thrombotic APS. In this review, we update the different clinical trials with NOACs in venous, arterial, and microvascular thrombosis and suggest how these patients should be managed in agreement with the expert panels. Although scarce data are published regarding the current role of NOACs in thrombotic APS, the clinical trials failed to demonstrate noninferiority of NOACs compared with VKA, especially in patients with triple antiphospholipid antibodies positivity and/or arterial thrombosis. Single or double antiphospholipid positivity should be analyzed on a case-by-case basis. In addition, we focus on different areas of uncertainty that still remain in thrombotic APS and NOACs. To summarize, emerging clinical trials are needed to provide robust data on the management of thrombotic APS.

This document focuses on the hematological treatment and prophylaxis that should be indicated in three groups of patients with hemophilia: Patients with inhibitors; Patients without inhibitors; and Patients receiving nonfactor replacement therapy.Inhibitors contribute to refractoriness to replacement treatment, leading to more severe bleeds. Immunotolerance and bypassing agents are essential to eradicate the inhibitors and bleeding control, respectively.Noninhibitors patients may receive standard half-life or extended half-life products, either on demand or prophylactically. Replacement factor concentrates are essential to control severe bleedings. Dose and frequency should be adjusted depending on the bleeding severity and type of factor concentrates (standard versus extended half-life products).Patients on emicizumab require bypassing agents (Novoseven is the agent of choice) in case of inhibitors and factor replacement in noninhibitor patients.

Emicizumab has improved the management of patients with hemophilia A with or without inhibitors. However, real-world data on drug plasma levels and their correlation with thrombin generation, viscoelastic clotting capacity and spontaneous joint annualized bleeding rate (SJABR) are scarce.