Etoposide-based treatment of adult HLH is associated with high biochemical response but poor survival outcomes.

Etoposide-based treatment is the standard of care for adult HLH in many centers, yet there remains a paucity of data regarding treatment outcomes. We conducted a retrospective study of 23 adults treated with etoposide-based therapy compared to 10 pediatric HLH cases at a single center. At diagnosis, the median serum ferritin was 20,071 µg/L and 937 µg/L in adults and children, respectively; median sIL-2r was 14,524 U/mL and 4,478 U/mL. Biochemical response to treatment was high, with 21/23 adults achieving >75% reduction in serum ferritin, but one year survival was only 7/21 compared to 7/10 in pediatric cases.

A case for consideration by apheresis practitioners: Melanoma and PD-1 inhibitor treatment in a patient with multiple relapses of immune thrombotic thrombocytopenic purpura.

Thrombotic Thrombocytopenic Purpura (TTP) is a rare life-threatening disease caused by ADAMTS-13 deficiency. Up to forty percent of patients with TTP relapse, and most relapse within eight years of their first presentation. This case report describes a patient with an aggressive course of TTP who subsequently developed metastatic melanoma while receiving prophylactic rituximab. Limited data exists regarding the potential for malignancy in patients receiving Rituximab for benign conditions. This is the first published case of melanoma in a TTP patient treated with rituximab. Melanoma treatment with PD-1 inhibitors is also associated with worsening of pre-existing autoimmune conditions. In this case, a splenectomy resulted in a durable remission despite treatment with PD-1 inhibitor.