Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen.

Acquired hemophilia A (AHA) is a rare severe autoimmune bleeding disorder with significant morbidity and mortality. Although critical for disease control, there is no consensus for the best immunosuppressive regimen. Most authors use steroids first line, followed by other agents for steroid failures. Upfront combined regimens offer the advantage of reduced steroid exposure and toxicity as well as increased efficacy. We retrospectively analyzed data from 32 patients with AHA treated on an identical such institutional protocol: cyclophosphamide 1000 mg on days 1 and 22, dexamethasone 40 mg on days 1, 8, 15, and 22, and rituximab 100 mg on days 1, 8, 15, and 22 (the regimen was termed CyDRi). All patients received at least 1 cycle of CyDRi. If necessary, CyDRi was repeated until remission, no sooner than day 43 of the previous cycle. Bleeding control was rapidly achieved. The median time for bleeding control was 15.5 days (range, 0-429 days; interquartile range, 2.5-29.5 days). Thirty-one (96.8%) of 32 patients achieved durable complete remission (CR); 29 (90.6%) of 32 patients were alive at last follow-up, all of them in CR. The median time to reach first CR was 77 days (range, 19-939 days; interquartile range, 31-115 days). Toxicity and side effects were acceptable and milder than those of commonly used, prolonged steroid therapies. In conclusion, the CyDRi regimen produced markedly higher CR rates and overall survival than currently used sequential regimens. Taken together, CyDRi proved to be an attractive option for the immunosuppression of elderly patients with AHA.

Usefulness of a Novel Electrocardiographic Score to Estimate the Pre-Test Probability of Acute Pulmonary Embolism.

According to our experience the 12-lead electrocardiogram (ECG) may be used to estimate the pretest probability of acute pulmonary embolism (acPE). To this end, we devised a novel ECG score (nECGs) composed of 5 known ECG criteria, best characterizing the key pathogenetic steps of acPE. A retrospective derivation cohort including 136 patients with acPE and a prospective validation cohort including 149 consecutive patients were used to devise and validate the nECGs. The latter cohort consisted of 76 patients with acPE and 73 controls presenting with characteristic symptoms of acPE, in whom the work-up ruled out acPE. We compared the diagnostic value of our nECGs with those of another ECG score (Daniel-ECG-score) and of the best prediction rules (3 Wells score and 2 Geneva score variants). The sensitivity (98.7%), negative predictive value (98%), test accuracy (84.4%) and the negative likelihood ratio (LR) (0.019) of the nECGs were superior to those of all other investigated methods. There was no between-groups difference in the positive LR. The specificity (69%) of the nECGs was inferior to those of the Daniel-ECG-score and Wells scores and did not differ or was superior to those of the Geneva score variants. The positive predictive value (77.3%) of the nECGs was superior to those of the 2 Geneva scores and did not differ from those of the other methods. In conclusion, the nECGs due to its superior sensitivity, negative predictive value, test accuracy, and negative LR estimated the pretest probability of acPE better than the Daniel-ECG-score and the prediction rules.