[Successful treatment of acquired hemophilia A with recombinant porcine factor VIII]. / Erfolgreiche Behandlung einer erworbenen Hemmkörperhämophilie A mit rekombinantem porcinem Faktor VIII.

Acquired hemophilia A is a rare, potentially life-threatening disease resulting from autoantibodies against coagulation factor VIII. We report the case of a patient with acquired hemophilia A and severe bleeding after incision of a peritonsillar abscess. Treatment with high dose factor VIII and recombinant activated factor VII failed to control bleeding. However, a single infusion of recombinant porcine factor VIII stopped bleeding efficiently and resulted in measurable factor VIII levels.

Durable responses to ibrutinib in patients with relapsed CLL after allogeneic stem cell transplantation.

Ibrutinib, a recently approved inhibitor of Bruton's tyrosine kinase (BTK), has shown great efficacy in patients with high-risk CLL. Nevertheless, there are few data regarding its use in patients who relapsed after allogeneic stem cell transplantation (alloSCT). We report clinical data from five CLL patients treated with ibrutinib for relapse after first or even second allogeneic transplantation. Additionally, we performed analyses on cytokine levels and direct measuring of CD4 Th1 and CD4 Th2 cells to evaluate possible clinically relevant immunomodulatory effects of ibrutinib. All patients achieved partial responses including one minimal residual disease (MRD)-negative remission. Within 1 year of follow-up, no relapse was observed. One patient died of severe pneumonia while on ibrutinib treatment. Beside this, no unexpected adverse events were observed. Flow cytometry and analyses of T cell-mediated cytokine levels (IL10 and TNFα) did not reveal substantial changes in T-cell distribution in favor of a CD4 Th1 T-cell shift in our patients. No acute exacerbation of GvHD was reported. In conclusion, these results support further evaluation of ibrutinib in CLL patients relapsing after alloSCT.