Management of thrombotic thrombocytopenic purpura without plasma exchange: the Jehovah’s Witness experience

George, James N.; Sandler, Steven; Stankiewicz, Joanna

doi:10.1182/bloodadvances.2017012351

Cited by 26 publications

(21 citation statements)

References 17 publications

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“…TTP is a severe disease with a high mortality that requires urgent diagnosis and initiation of effective treatment, especially the plasma exchange . The hall marker of TTP is a severe ADAMTS13 deficiency .…”

Section: Discussionmentioning

confidence: 99%

A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score

Zhao

Zhou

et al. 2019

J of Clinical Apheresis

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Background: The PLASMIC score was recently published to aid in the early identification of thrombotic thrombocytopenic purpura (TTP) patients. This study aims to evaluate whether this score is suitable for Chinese suspected TTP patients and find the utility of patients' other characteristics in predicting severe ADAMTS13 deficiency. Methods: We retrospectively studied a Chinese cohort of 38 consecutive hospitalized patients with suspected TTP, ADAMTS13 test results, and other clinical data from September 2016 to May 2018. The predictive power of PLASMIC score in our cohort was evaluated, and patients' other characteristics, especially the high lactate dehydrogenase/the upper limit of normal (LDH/ULN), were studied to determine their distinguishing ability for TTP patients.Results: In this Chinese cohort, 17 patients were diagnosed with TTP according to ADAMTS13 activity results. When dichotomized at intermediate-high risk (scores 5-7) vs low risk (scores 0-4), the PLASMIC score predicted TTP with a sensitivity of 100%, a specificity of 9.52%, and a misdiagnosis rate of 90.48%. And the LDH/ULN alone, or plus platelet count, reticulocyte percentage and indirect bilirubin (IBIL) both had excellent predictive power (area under the curve [AUC] 0.937, 95% confidence interval [CI] 0.863-1.000, P = .000, and AUC 0.994, 95% CI 0.980-1.000, P = .000, respectively). The model including platelet count, reticulocyte percentage, IBIL, and LDH/ULN ratio had a sensitivity of 100%, a specificity of 95.2%, and a misdiagnosis rate of 4.8%.Conclusions: A modified PLASMIC score plus LDH/ULN ratio might be more suitable for identifying ADAMTS13 deficiency patients, especially for making an earlier diagnosis, guiding the immediate and reasonable plasma exchange, and also avoiding unnecessary allocation of plasma. K E Y W O R D SADAMTS13 activity, lactate dehydrogenase, PLASMIC score

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Section: Discussionmentioning

confidence: 99%

A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score

Zhao

Zhou

et al. 2019

J of Clinical Apheresis

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“…However, this presents a unique challenge in individuals who are unable to accept plasma due to religious beliefs. With Jehovah's Witnesses, there have been 8 published case reports discussing the successful management of TTP (7 idiopathic, 1 drug related) without the use of TPE (2)(3)(4)(5)(6)(7)(8)(9). There is likely a publication bias with only successful cases being reported.…”

Section: Introductionmentioning

confidence: 99%

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

Lim

Greenberg

2019

Journal of Patient Experience

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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.

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“…To our knowledge, there have been only five published case reports of Jehovah's Witnesses whose diagnosis of aTTP is confirmed or considered likely (Table ) . Of the eight episodes in these five and our case, plasma exchange with human albumin solution as the replacement fluid was performed in five episodes.…”

Section: Discussionmentioning

confidence: 70%

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

Baseri

Vishwanathan

Benasher

et al. 2019

J of Clinical Apheresis

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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.

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Management of thrombotic thrombocytopenic purpura without plasma exchange: the Jehovah’s Witness experience

Abstract: Key Points TTP in Jehovah’s Witness patients has been managed successfully without PEX. This experience, plus new TTP treatments, may make it possible for patients who are not Jehovah’s Witnesses to avoid PEX in the future.

Cited by 26 publications

References 17 publications

A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score

A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score

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

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

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