| Literature DB >> 31116457 |
Babak Baseri1,2, Swati Vishwanathan1,2, Daniel Benasher3, Mikhail Khazan2, Carol Luhrs1, Han-Mou Tsai1.
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.Entities:
Keywords: zzm321990N-acetylcysteine; Jehovah's Witness; bortezomib; cryoprecipitate; factor VIII concentrates; rituximab; thrombotic thrombocytopenic purpura
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Year: 2019 PMID: 31116457 DOI: 10.1002/jca.21710
Source DB: PubMed Journal: J Clin Apher ISSN: 0733-2459 Impact factor: 2.821