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Evans' syndrome: possible benefit from plasma exchange

E Patten, F P Reuter

    Transfusion
    |September 1, 1980
    PubMed
    Summary

    Plasma exchange can be a life-saving treatment for Evans' syndrome, a rare autoimmune disorder. This therapy significantly improved a patient's hematocrit and platelet counts when other treatments failed.

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    Area of Science:

    • Hematology
    • Immunology
    • Plasma Exchange Therapy

    Background:

    • Evans' syndrome is a rare autoimmune disorder characterized by immune thrombocytopenia and autoimmune hemolytic anemia.
    • Standard treatments including splenectomy, corticosteroids, immunosuppressants, and transfusions can be ineffective in severe cases.

    Observation:

    • A 45-year-old female patient with refractory Evans' syndrome was treated with plasma exchange.
    • The patient had a critically low hematocrit and platelet count despite aggressive conventional therapies.

    Findings:

    • Nine liters of plasma were exchanged over 12 days.
    • Following plasma exchange, hematocrit increased from <16% to 29%, and platelet count rose from 14,500/microliter to 272,000/microliter.
    • Transfusion requirements decreased significantly post-procedure.

    Implications:

    • Plasma exchange is a viable therapeutic option for severe, refractory Evans' syndrome.
    • This treatment can rapidly improve hematological parameters and reduce the need for blood transfusions.
    • Further investigation into plasma exchange for similar autoimmune hematological conditions is warranted.

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