Therapeutic modalities in thrombotic thrombocytopenic purpura management among Jehovah's Witness patients: A review of reported cases

Abstract

Introduction: Devout members of the Jehovah's Witness faith flatly refuse transfusions of white blood cells, red blood cells, platelets, and plasma. The latter agent is a mainstay in the treatment of thrombotic thrombocytopenic purpura (TTP). Alternative treatment options for Jehovah's Witness patients are needed and reviewed herein.

Methods: Cases of TTP treatment among Jehovah's Witnesses were obtained from the published literature. Key baseline and clinical data were extracted and summarized.

Results: A total of 13 reports spanning a 23-year period and 15 TTP episodes were identified. Median (IQR) age was 45.5 (29.0-57.5) and 12/13 (93%) patients were female. Neurologic symptoms were present in 7/15 (47%) episodes at presentation. Disease confirmation with ADAMTS13 testing was present in 11/15 (73%) of episodes. Corticosteroids and rituximab were employed in 13/15 (87%) and 12/15 (80%) of cases, respectively, with apheresis-based therapy employed in 9/15 (60%) episodes. For eligible cases, caplacizumab was used in 4/5 (80%) episodes; average time to platelet response was shortest in these cases. Sources of exogenous ADAMTS13 accepted by patients in this series included cryo-poor plasma, FVIII concentrate, and cryoprecipitate.

Conclusions: Successful management of TTP within the boundaries of the Jehovah's Witness faith is possible.

Keywords: Caplacizumab; Jehovah’s Witness; Koate; Rituximab; TTP; Thrombotic Thrombocytopenic Purpura.