Objective: Acquired hemophilia A (AHA) is a rare autoimmune disease characterized by the presence of autoantibodies against coagulation factor VIII, leading to spontaneous hemorrhage in patients without a prior family or personal history of bleeding. This study describes the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcomes of 41 AHA patients together with specific case reports. Materials and Methods: Diagnosis and treatment of these patients occurred between 2005 and 2023. The median age at diagnosis was 67.8 (range: 15-93) years. Among the 41 patients, 10 (24%) cases were idiopathic, 4 (10%) were postpartum, 18 (44%) involved autoimmune diseases, and 9 (22%) involved a diagnosis of cancer. Results: The diagnostic delay exceeded 30 days in 15 of the 41 cases (36.5%). A total of 38 of the 41 (93%) patients presented with spontaneous bleeding, with mucocutaneous bleeding being the most common presentation (23/41, 56%). Four patients experienced postpartum bleeding. Clinical remission was achieved by 100% of patients and no patients died. Conclusion: Hemostatic and immunosuppressive therapy is essential in AHA, and it should be started as soon as possible in patients with bleeding. However, a significant delay in diagnosis was observed in these cases. The absence of mortality is likely attributable to the management of the disease within a specialized hemostasis and thrombosis unit, which offers a clinical ward, a specialized laboratory, and a dedicated ambulatory service. The Italian Society for the Study of Haemostasis and Thrombosis is working to secure recognition of this essential role in every hospital.

Background: Patient's adherence to oral anticoagulant therapy is essential to prevent and treat thrombotic events. Methods: To assess the patients' adherence Morisky Medication Adherence Scale 8-items was used. The target population included 785 consecutive outpatients, of whom 384 were on Vitamin K Antagonists and 401 on Direct Oral Anticoagulants. Moreover, we evaluate which variable among age, gender, having experienced a thrombotic episode, time in the therapeutic range for patients on Vitamin K Antagonists, being naive and once versus twice daily drug assumption for patients on Direct Oral Anticoagulants, could affect adherence to therapy. Results: Morisky Medication Adherence Scale 8-items score was 8 in both groups. The intentional non-adherence obtained the lowest score while the unintentional non-adherence is the most frequent problem in patients treated with either Vitamin K Antagonists or Direct Oral Anticoagulants. Age > 75 years, male gender, having experienced a thrombotic episode, being naive and assuming Direct Oral Anticoagulants twice a day were signifi-cantly associated with a higher risk to forget assuming the oral anticoagulant, to have more difficulty in remembering to take it or to bring it in case of travel or leaving home. A low percentage of time in therapeutic range was associated with a not regularly assumption of the anticoagulants. Conclusion: Patients treated with Vitamin K Antagonists or Direct Oral Anticoagulants show a good adherence and persistence to their oral anticoagulant therapy. Several factors have been identified to affect patients' adherence and deserve a careful attention by the doctors at the Anticoagulation Clinic. (c) 2021 Elsevier B.V. All rights reserved.

Point-of-care testing (POCT) devices can be used to monitor anticoagulant therapy. We compared patients being monitored at home by self-testing using a POCT device and telemedicine support with a previous period of conventional monitoring at a Thrombosis Centre. A total of 114 anticoagulated patients participated. The number of blood checks (INR) was significantly higher in the home monitoring group and the interval between checks was significantly shorter. The percentage of missed INR checks was significantly higher during the conventional monitoring period compared with home monitoring. Patients were divided into two groups on the basis of the time spent within the therapeutic range (TTR) during conventional monitoring: the unstable group had TTR<70% and the stable group had TTR ≥70%. In the unstable group there was a significant increase in TTR with home monitoring: 63% to 68% (P < 0.001) while in the stable group there was no significant change (77% to 75%). The study showed that oral anticoagulation management by means of self-testing is suitable and safe.

Introduction: Point-of-care testing (POCT) prothrombin time monitors are now widely used to monitor oral anticoagulant treatment. Although portable coagulometers are extremely easy to use, checking the quality of their performance presents some difficulties.Materials and Methods: The aims of this study were to investigate on a quarterly basis the performance of 95 Coagucheck S assigned to 99 anticoagulated patients at home. This was done checking the monitors versus a reference coagulometer in the laboratory at our Thrombosis Centre (TC). The other aims were to carry out an external quality assessment employing different sets of INR certified plasmas with 5 different ranges of anticoagulation and to assess the performance of the different lots of strips employed by the patients during the study.Results: No difference between the PT INR obtained with both the systems at the first quarterly check was noted but a significant difference was found when the two systems were compared at the second and third quarterly checks. The Bland-Altman test showed increased disagreement between the first and the third controls. The percentage of INR values that showed a difference of more or less than 0.5 INR units in the PT values performed with both the systems was: 1.0% (first control), 7.5% (second control) and 11.5% (third control) (Chi-Square: 8.315, p=0.0156). Lots with differences higher than 10% in terms of 0.5 INR Units at the first, second and third controls were 16%, 20.8% and 61%, respectively. Seven monitors (7.3%) failed to test one or two of the INR certified plasmas of one set but performed well using a second set of plasmas. Three monitors (3.1%) failed to test two sets of plasmas but performed well using a different lot of strips (from 279A to 483A). One monitor (11%) gave unsatisfactory results with different sets of plasmas and strips. All the other PT INR obtained with the monitors fell well within the different ranges of the INR certified plasmas.Conclusions: Anticoagulated patient in self-testing or self-management should periodically bring their portable coagulometer to a reference Thrombosis Centre especially when the lot of strips have to be changed. The role of Thrombosis Centre appears therefore crucial in this regard. (C) 2008 Elsevier Ltd. All rights reserved.