Abstract | PURPOSE OF REVIEW: RECENT FINDINGS: Studies indicate that reducing anti-ADAMTS13 antibody levels through B-cell depletion or proteasome inhibition is effective for the management of refractory disease. Preliminary reports examining anti-CD20 therapy for the treatment of initial disease or as maintenance therapy for seropositive patients suggest the addition of immunosuppression in other disease phases may delay relapse. Exciting developments in targeted therapies to von Willebrand Factor and recombinant ADAMTS13 hold promise for transforming disease management. SUMMARY: Approximately half of patients diagnosed with acquired thrombotic thrombocytopenic purpura experience refractory and/or relapsing disease. For these patients, a hematologic remission may be an insufficient therapeutic goal. With recent developments, it is now possible to envision a multifaceted approach targeting disease mechanisms that may dramatically improve outcomes for this otherwise debilitating disease.
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Authors | Yvette C Tanhehco, Gowthami Arepally, Ara Metjian |
Journal | Current opinion in hematology
(Curr Opin Hematol)
Vol. 24
Issue 6
Pg. 521-528
(Nov 2017)
ISSN: 1531-7048 [Electronic] United States |
PMID | 28759473
(Publication Type: Journal Article, Review)
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Chemical References |
- Autoantibodies
- Steroids
- ADAMTS13 Protein
- ADAMTS13 protein, human
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Topics |
- ADAMTS13 Protein
(blood)
- Autoantibodies
(blood)
- Humans
- Immunosuppression Therapy
(methods)
- Plasma Exchange
(methods)
- Purpura, Thrombotic Thrombocytopenic
(blood, therapy)
- Steroids
(therapeutic use)
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