Successful Management of Thrombotic Thrombocytopenic Purpura in a Jehovah’s Witness: An Individualized Approach With Joint Decision-Making

Abstract

The management of thrombotic thrombocytopenic purpura (TTP) presents a unique challenge in individuals who are unable to accept plasma due to religious beliefs, given that therapeutic plasma exchange (TPE) is the standard of care. A 61-year-old Jehovah’s Witness woman presented to our hospital with neurological symptoms and laboratory findings suggestive of TTP. On admission, she refused transfusion of blood products, specifically red blood cells, platelets, and plasma but accepted albumin and intravenous immunoglobulin (IVIG); fractions of plasma. She was started on steroids, IVIG, and TPE with albumin as replacement therapy with minimal improvement. After a detailed discussion with the patient and family, they agreed to accept cryosupernatant. The patient started TPE with cryosupernatant for replacement therapy, which resulted in clinical improvement. This case highlights the importance of an individualized approach with joint decision-making given the significant heterogeneity that exists in Jehovah’s Witnesses’ attitude toward the receipt of blood products.

Treating thrombotic thrombocytopenic purpura without plasma exchange during the COVID-19 pandemic. A case report and a brief literature review

“…However, it is important to note that plasma extraction was performed and replaced with human albumin solution [ 5 ]. Similar cases of TTP in Jehova’s Witnesses treated without PEx using corticosteroids, rituximab, and albumin as a replacement fluid in combination with different drugs have also been reported ( Table 2 ) [ [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] ]. In this scenario, it is difficult to evaluate the contribution of plasma extraction or fluid replacement alone versus the use of rituximab and corticosteroids on the good response obtained by those patients.…”

Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura

“…In literature, there are several experiences with different therapeutic approaches that demonstrates the non‐criticality of plasma usage. For Jehovah's Witness who refuse PEX therapy, interesting results 10,15‐19 have been achieved with the use of albumin or other proteins, or employing caplacizumab and immunosuppression 19 . In the Hercules trial, in which caplacizumab added to the standard PEX + steroids treatment was compared with standard treatment alone, 43% of pts.…”

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

“…As TPE is the foundation of management of acute episodes, this presents a unique challenge in the management of these patients. Various regimens have previously been tried, including vincristine [ 192 ] and plasma exchange with albumin [ 193 ] or cryosupernatant [ 194 ] replacement. With the use of caplacizumab alongside improved immunosuppressive therapy, successful treatment without TPE has been described not only in this patient population [ 185 ] but also in other selected patients, including one with anaphylaxis to plasma [ 186 , 187 , 188 ].…”