Eculizumab in Transplant-Associated Thrombotic Microangiopathy

Clinical and Applied Thrombosis/Hemostasis - Tập 23 Số 2 - Trang 175-180 - 2017
Prajwal Dhakal1, Smith Giri2, Ranjan Pathak3, Vijaya Raj Bhatt4
11 Department of Medicine, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal.
22 Department of Medicine, University of Tennessee Health Science Center, Memphis, TN, USA.
33 Department of Medicine, Reading Health System, West Reading, PA, USA.
44 Department of Internal Medicine, Division of Hematology-Oncology, University of Nebraska Medical Center, Omaha, NE, USA.

Tóm tắt

Introduction: Transplant-associated thrombotic microangiopathy (TA-TMA) is a rare entity with no standard of care and high mortality, despite the use of plasma exchange. Methods: Using specific search terms, all cases having TA-TMA treated with eculizumab and indexed in MEDLINE (English language only) by November 2014 were reviewed. Results: A total of 26 cases, 53% men, had a median age of 33 years (range 2-61). Transplant-associated thrombotic microangiopathy occurred after stem-cell transplant (35%) or solid-organ transplant (65%), frequently associated with the use of cyclosporine or tacrolimus (96%). A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS 13) level was always >10%. After TA-TMA diagnosis, the following drug adjustments were made: discontinuation of cyclosporine or tacrolimus in 45%, dose reduction in another 27%, continuation of the drugs in 23%, and switch from cyclosporine to tacrolimus in remaining 5%. Plasma exchange was performed in ∼43%. The median interval between transplant and initiation of eculizumab was 63 days (range 11-512). A median of 5.5 doses (range 2-21) of eculizumab was utilized with 92% response occurring after a median of 2 doses (range 1-18). At a median follow-up of 52 weeks (range 3-113), the survivors (92%) were doing well. Conclusion: Within the limits of this retrospective analysis, our study demonstrates that eculizumab use may result in high response rate and 1-year survival in patients with TA-TMA refractory to discontinuation of calcineurin inhibitor and plasma exchange.

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Clinical and Applied Thrombosis/Hemostasis

Tập 23 Số 2

175-180

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