Outpatient Follow Up

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VISITS AND STANDARD LABS

After discharge, we recommend weekly clinical evaluations and lab visits for a month or while tapering steroids. Routine labs for monitoring include CBC with differential, comprehensive metabolic panel, reticulocyte count and LDH. Afterwards clinic and lab visits should be conducted monthly for 5 months followed by every 3 months thereafter.

ADAMTS-13 activity testing

If the patient is on Caplacizumab, (1-2) The USTMA recommends checking ADAMTS-13 activity weekly.

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If the ADAMTS-13 has increased > 10% in 2 consecutive weeks or > 30% in one occasion then, Caplacizumab can be discontinued. We suggest checking ADAMTS-13 activity weekly for 1-month followed by monthly for 3 months and then every 3 months subsequently.

If the ADAMTS-13 remains < 10% after completion of 28 days of Caplacizumab, USTMA suggests considering extending Caplacizumab for an additional 4 weeks and intensifying immunosuppression with Rituximab(3), if not already given, or with cyclosporine, cyclophosphamide, bortezomib, vincristine or mycophenolate. The choice of immunosuppressive therapy is based upon several factors, including the patient’s age, other comorbidities (including renal failure), drug access, and the physician’s comfort level and experience.

ADAMTS-13 activity should be checked weekly for a month, monthly for 3 months and then every 3-month interval thereafter.

References

1. Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016; 374:511-522

2. Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019; 380:335-346

3. McDonald V, Manns K, Mackie IJ, Machin SJ, Scully MA.Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpura. J Thromb Haemost. 2010 Jun; 8(6):1201-8.

4.Jestin M, Benhamou Y, Schelpe AS, et al. Preemptive rituximab prevents long term relapses in immune-mediated thrombotic thrombocytopenic purpura. Blood. 2018;132:2143-2153