Survival of a Jehovah's Witness with thrombotic thrombocytopenic purpura without using plasma: A case report and review of the literature

Abstract

Acquired thrombotic thrombocytopenic purpura (aTTP) is a serious disorder with arteriolar and capillary thrombosis for which the treatment usually requires plasma exchange with plasma as the replacement fluid. Management of patients who do not accept blood products is a serious challenge. We present the case of a Jehovah's Witness patient who achieved clinical response after treatment with plasma exchange using human albumin solution as the replacing fluid, high dose corticosteroids, and rituximab. The patient also received ADAMTS13 containing plasma cryoprecipitate and von Willebrand factor VIII concentrates. She had an exacerbation of her TTP in less than 3 weeks. She was treated with further plasma exchange with human albumin solution as the replacement fluid, high dose steroids, and rituximab. Bortezomib and N-acetylcysteine were added. The patient eventually improved clinically and achieved remission that is ongoing for more than 7 months. A review of the literature shows that all five previously reported cases of aTTP in Jehovah's Witnesses survived although none received plasma. Two were not even treated with plasma exchange. The experience of this case and those in the literature demonstrates that remission of aTTP may be achieved without using plasma or plasma exchange.

Bortezomib, a promising alternative for patients with refractory or relapsed thrombotic thrombocytopenic purpura after rituximab treatment

“…4 We summarized nine refractory or relapsed TTP cases in Table 2 after Eskazan's report, who were unresponsive to rituximab and then transferred to bortezomib treatments. [3][4][5][6][7][8][9] Putting these cases together with ours, 25 patients with relapsed or refractory TTP were treated with bortezomib. The age distribution of these patients ranged from 5 to 76 years, suggesting that bortezomib could be used not only in adult, but also in the elderly 10 and children.…”

Spontaneous recovery in a patient with acquired thrombotic thrombocytopenic purpura (TTP): observation of a ‘subclinical’ TTP state

“…PLEX is initiated urgently to remove or dilute the ADAMTS13 inhibitor autoantibodies, replace deficient ADAMTS13 enzyme, and regulate the size of vWF multimers to diminish thrombotic risk [14]. While PLEX is the cornerstone of TTP management, studies have reported remissions in patients treated without PLEX [9,10,15,16]. These populations, predominantly comprised of Jehovah's Witnesses, received other therapeutic interventions such as plasmapheresis with albumin rather than plasma replacement, corticosteroids, rituximab, other chemotherapeutic agents, and most recently the anti-vWF nanobody caplacizumab.…”

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.…”