Efficacy of therapeutic plasma exchange in a patient with coagulation inhibitors (acquired haemophilia A) – A case report

Kumari, Sangeeta; Tripathi, Parmatma Prasad; Sharma, Ratti Ram; Hans, Rekha; Lamba, Divjot Singh; Khadwal, Alka; Singh, Charanpreet

doi:10.1016/j.transci.2020.102809

Cited by 3 publications

(2 citation statements)

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“…Moreover, a recent case report demonstrated that TPE resulted in complete recovery of life-threatening bleeding in a patient with AHA who was non-responsive to immunosuppressant therapy and had high titers of coagulation inhibitors. [14] Nevertheless, the role of plasma exchange in the treatment of AHA is unclear. Moreover, the first-line rFVIIa or aPCC treatments (hemostatic bypassing agents) for AHA are either, not easily available, or unaffordable for patients in third world countries, especially in West China.…”

Section: Introductionmentioning

confidence: 99%

“…In TTP, TPE is used to quickly remove the inhibitor, supply protease enzymes (ADAMSTS), and then these patients can achieve remission with rituximab and immunosuppressants. Moreover, a recent case report demonstrated that TPE resulted in complete recovery of life-threatening bleeding in a patient with AHA who was non-responsive to immunosuppressant therapy and had high titers of coagulation inhibitors [14] . Nevertheless, the role of plasma exchange in the treatment of AHA is unclear.…”

Section: Introductionmentioning

confidence: 99%

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Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A

Wang¹,

Shen²,

Zeng³

et al. 2021

Medicine

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Poor availability and a lack of affordability of bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) in west China prompted us to investigate an alternative cost-effective combination therapy. We aimed to explore the feasibility of therapeutic plasma exchange (TPE)-based combination therapy in the treatment of acquired hemophilia A (AHA).We retrospectively investigated the clinical features of AHA in 6 patients who were treated with a combination of TPE, corticosteroids, and rituximab in our department for 9 years between January, 2011 and December, 2019.We examined 1 male and 5 female patients. The median age at diagnosis of AHA was 51 years (18-66 years). In all patients, FVIII activity levels were low (median: 1.5%; 1-3%), FVIII inhibitor titers were high (median: 24.5 BU/mL; 13.2-48.6 BU/mL), and activated partial thromboplastin time was markedly prolonged (median: 99.4 s; 60.9-110.1 s). They underwent 2 to 8 cycles of plasma exchange and were given varying combinations of dexamethasone, methylprednisolone, prednisone, and rituximab. After TPE bleeding gradually stopped, and activated partial thromboplastin time decreased. After 3 months of treatment, FVIII inhibitors completely disappeared.TPE when combined with corticosteroids and rituximab, as adjunctive immunosuppressive agents, may be an effective and reliable treatment for AHA. When there is no alternative, intensive first-line treatment including TPE may be lifesaving.Abbreviations: AHA = acquired hemophilia A, aPTT = activated partial thromboplastin time, CR = complete remission, MDS = myelodysplastic syndrome, PR = partial remission, RBC = red blood cell, rFVIIa = recombinant factor VIIa, rpFVIII = recombinant porcine FVIII, SLE = systemic lupus erythematosus, TPE = therapeutic plasma exchange.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A

Wang¹,

Shen²,

Zeng³

et al. 2021

Medicine

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Multiple drugs

2020

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Effective Management of a Patient with Acquired Coagulation Factor Inhibitors Using Therapeutic Plasma Exchange Alone

Ryu

Kim

et al. 2021

Lab Med Online

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Acquired coagulation factor inhibitors are autoantibodies that mainly inhibit the action of coagulation factors, and prompt diagnosis and treatment are important in this situation. Treatment often consists of suppression of hemorrhage, elimination of autoantibodies, and correction of the underlying cause. Therapeutic plasma exchange (TPE) is used as an adjuvant treatment in patients with acquired coagulation factor inhibitors; however, there is no consensus about its clinical significance. Herein, we reported a case of a 70-year-old female patient with acquired coagulation factor inhibitors treated with 13 sessions of TPE after antibiotic treatment. The presence of inhibitors was confirmed by the increased coagulation times without correction in the mixing tests and a factor VIII inhibitor level of 12 Bethesda U/mL. Since the patient could not undergo conventional immunosuppressive therapy because of suspected infective endocarditis and spinal osteomyelitis, only TPE was performed with a successful outcome. To our knowledge, this is the first case where acquired coagulation factor inhibitors were managed by TPE alone. Therefore, TPE might be an effective treatment option in patients with acquired coagulation factor inhibitors when conventional immunosuppressive therapy cannot be applied.

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Efficacy of therapeutic plasma exchange in a patient with coagulation inhibitors (acquired haemophilia A) – A case report

Cited by 3 publications

References 4 publications

Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A

Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A

Multiple drugs

Effective Management of a Patient with Acquired Coagulation Factor Inhibitors Using Therapeutic Plasma Exchange Alone

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