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

Lim, Ming Y.; Greenberg, Charles S.

doi:10.1177/2374373519829902

Cited by 7 publications

(6 citation statements)

References 21 publications

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“…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.…”

Section: Discussionsupporting

confidence: 54%

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

Galindo-Calvillo¹,

Rodríguez-Roque²,

León

et al. 2021

Transfusion and Apheresis Science

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We report the case of a patient diagnosed with a clinical relapse of acquired immune-mediated thrombotic thrombocytopenic purpura (TTP) who was successfully treated with low-dose rituximab plus corticosteroids without the use of plasma exchange (PEx), which was unavailable at the time due to the COVID-19 pandemic. Rituximab 100 mg weekly for 4 weeks was administered, combined with 1 mg/kg of prednisone, obtaining a complete hematological response in 6 weeks. This case suggests that PEx may be unnecessary for a subset of patients with relapsed TTP who are clinically stable without significant end-organ damage. A brief literature review regarding TTP patients treated without plasma exchange is also included.

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Section: Discussionsupporting

confidence: 54%

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

Galindo-Calvillo¹,

Rodríguez-Roque²,

León

et al. 2021

Transfusion and Apheresis Science

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Perrone

Passucci

Barbera

et al. 2021

J of Clinical Apheresis

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Management of aTTP in patients who refuse or are intolerant to plasma remains challenging, but new drugs can be implemented with success. A 39‐year‐old woman presented to the Emergency department for bruises at the upper and lower limbs together with worsening anemia and thrombocytopenia; PLASMIC score was seven, indicative of high risk to have a thrombotic microangiopathy due to severe ADAMTS‐13 deficiency: indeed, it was 1.4%. We immediately started Plasma Exchange, but after the third procedure she developed severe anaphylaxis to Octaplas plasma, so PEXs were discontinued. We proceeded to a salvage strategy with rituximab and caplacizumab that was rapidly effective to resolve symptoms and hemolysis. It has been already reported a case in which a patient developed severe reactions to fresh‐frozen plasma that required discontinuation of PEX. Differently from this case, our patient was already using the less immunogenic pooled plasma units Octaplas, therefore a strategy with caplacizumab was the only available option. Moreover, rituximab is associated with a shorter time to obtain a durable remission in aTTP and a faster time (15 days) to final ADAMTS13 activity recovery >10%. To our knowledge, this is the first case of early discontinuation of caplacizumab in a patient allergic to PEX by actively monitoring ADAMTS13 activity, allowing optimization of healthcare resources during COVID‐19 pandemic.

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“…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 ].…”

Section: Special Populationsmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

Sukumar

Lämmle

Cataland

2021

JCM

125

132

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Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTTP) is often detected in childhood or during pregnancy. iTTP occurs more often in women and is potentially lethal without prompt recognition and treatment. Front-line therapy includes daily plasma exchange with fresh frozen plasma replacement and immunosuppression with corticosteroids. Immunosuppression targeting ADAMTS13 autoantibodies with the humanized anti-CD20 monoclonal antibody rituximab is frequently added to the initial therapy. If available, anti-VWF therapy with caplacizumab is also added to the front-line setting. While it is hypothesized that refractory TTP will be less common in the era of caplacizumab, in relapsed or refractory cases cyclosporine A, N-acetylcysteine, bortezomib, cyclophosphamide, vincristine, or splenectomy can be considered. Novel agents, such as recombinant ADAMTS13, are also currently under investigation and show promise for the treatment of TTP. Long-term follow-up after the acute episode is critical to monitor for relapse and to diagnose and manage chronic sequelae of this disease.

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

Cited by 7 publications

References 21 publications

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

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

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

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

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