How and when to treat thrombocytopenia in SLE

How and when to treat thrombocytopenia in SLE?

  • Treatment is recommended for platelet count <30,000/ mm or with significant bleeding.
  • • Glucocorticoids (dexamethasone or prednisone): consider pulse steroids for severe disease
  • • Splenectomy: should not be first line, may be associated with increased risk of clot and sepsis
  • • IVIG: 2 g/kg (400 mg/kg/day × 5 days). Very effective therapy to raise platelet counts acutely. This treatment can be used in preparation for splenectomy or if the patient showed signs of bleeding. Because of its cost, however, repeated treatments with IVIG are not a reasonable long-term therapeutic option.
  • • For Rh-positive nonsplenectomized patients, anti-D (RhD) can be used.
  • • Rituximab: can induce a durable response, but the rate of response at >1 year is only 18% to 35%.
  • • Thrombopoietin receptor agonists (romiplostim/eltrombopag/avatrombopag): considered in patients who have failed glucocorticoids; however, they are expensive ($85,000/year) and thrombocytopenia usually recurs when the drug is stopped.
  • • Second-line agents:
    • • AZA
    • • MMF
    • • Cyclosporine or tacrolimus
    • • Danazol: an androgen that increases platelet counts and allows steroid dose to be decreased. Doses up to 800 mg/day may be necessary. The androgenic side effects in a young female may be troubling.
    • • CYC
    • • Fostamatinib
    • • Others: dapsone, vincristine, combination of drugs

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