Emicizumab for acquired hemophilia A: Report of two cases and dosing strategies.

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by autoantibodies against FVIII. Severe AHA is life-threatening. Currently, licensed hemostatic agents for the treatment of severe AHA have short half-lives and require intravenous administration, leading to a need for hospitalization, higher costs, and negative effects on quality of life. We present two cases of severe AHA with high inhibitor titers where emicizumab was safely and effectively used with intensive immunosuppression. These reports suggest in vivo efficacy even in high inhibitor environments. The optimal dosing regimen (accelerated vs. standard loading, maintenance frequency) is unknown and we discuss the current approaches.

Disparities in Outcomes of Hospitalizations Due to Multiple Myeloma: A Nationwide Comparison.

Background With the advent of novel treatments, there is a declining trend in the multiple myeloma (MM) mortality rate with an increasing hospitalization rate. However, there is limited population-based data on trends and outcomes of hospitalizations due to MM in the United States (US). Methods We analyzed the publicly available Nationwide Inpatient Sample (NIS) from 2007 to 2017 to identify MM hospitalizations. Results Hospitalizations for MM increased from 17,100 (8.71%) in 2007 to 19,490 (9.92%) in 2017. The in-hospital mortality rate declined from 8.4% in 2007 to 4.9% in 2017 (P <0.001) and discharge to facilities decreased from 20.4% in 2007 to 17.4% in 2017 (P <0.001). The odds of in-hospital mortality were higher with increasing age (odds ratio (OR): 1.46; 95% confidence interval (CI): 1.38 -1.54; P <0.0001), pneumonia (OR: 4.18; 95% CI: 3.63 - 4.81, P <0.0001), septicemia (OR: 2.50; 95% CI: 2.22 - 2.82; P <0.0001), renal failure (OR: 1.48; 95% CI: 1.34 -1.64; P <0.0001), uninsured/self-pay insurance status (OR: 2.69; 95% CI: 2.18 - 3.3; P <0.0001), rural hospital (OR: 2.26; 95% CI: 1.88 -2.72; P<0.0001), and urban-non-teaching hospitals (OR: 1.38; 95% CI: 1.23 - 1.56; P <0.0001). Also, increasing age (OR: 1.14; 95% CI: 1.11-1.18, P <0.0001), Black race (OR: 1.12; 95% CI: 1.02-1.23, P <0.0001), and multiple comorbidities were associated with higher disability. Conclusion Hospitalizations for MM continued to increase, whereas in-hospital mortality continued to decrease. Advanced age, sepsis, pneumonia, and renal failure were associated with higher odds of mortality in MM patients.