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ITP: The Past Decade 2006-2015 Ming Hou Qilu Hospital, Shandong University.

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Presentation on theme: "ITP: The Past Decade 2006-2015 Ming Hou Qilu Hospital, Shandong University."— Presentation transcript:

1 ITP: The Past Decade 2006-2015 Ming Hou Qilu Hospital, Shandong University

2 Nomenclature Idiopathic Thrombocytopenic Purpura (ITP) Immune Thrombocytopenia (ITP) 2007 International ITP working group. Blood. 2009, 113(11):2386-93.

3 Phases of ITP International ITP working group. Blood. 2009, 113(11):2386-93. 0 6 Months Chronic ITP Acute ITP 2007 0 3 Months 12 Months Newly ITPPersistent ITPChronic ITP

4 USA UK Japan Epidemiology and incidence of ITP France Danmark

5 Pathogenesis of ITP Decreased production Increased destruction Humoral immunity Cell immunity Spleen RES Liver Desialya tion CTL lysis Liver Desialya tion J Immunol. 2015 Jun 15;194(12):5579-5587. Br J Haematol. 2014 Apr;165(2):248-58 Loss of Immune tolerance Dysmegakaryocyt opoiesis Differe ntiation Aapop tosis

6 Loss of T cell tolerance Anergy T cell Tregs Apoptotic T cell Th1/Th2

7 T cell-mediated cytotoxicity

8 Dysmegakaryocytopoiesis

9 Desialyation Hepatocytes PLT Platelet desialylation Qiu J, ASH Annual Meeting Abstracts, 2014 Ni H, Nat Communication, 2015

10 Laboratory Diagnosis of ITP MAIPA/Flow Cytometric Immunobead Assay immune vs non-immune TPO concentration assay ITP vs atypical AA or MDS Curr Hematol Rep. 2005 Mar;4(2):160-5. Br J Haematol. 2009 Sep;146(6):585-96. Eur J Haematol. 2011 Apr;86(4):339-46. Br J Haematol. 1998 Jun;101(3):420-4.

11 Treatment not indicated platelet > 30×10 9 /L no bleeding symptom no increased risk of bleeding International ITP working group. Blood. 2009, 113(11):2386-93. ITP therapy

12 Conventional PDN HD-DXM International ITP working group. Blood. 2009, 113(11):2386-93. First-line treatment short-term application

13 Conventional PDN vs HD-DXM One or two courses of HD-DXM demonstrated higher CR rate, shorter time to response and less adverse events than PDN. Wei Y, et al, Conventional Oral Prednisone Versus High-Dose Dexamethasone for Management of Adult Immune Thrombocytopenia: A Prospective Randomized Multicenter Clinical Trial, 2014 ASH abstract, 1455 NCT01356511

14 Thrombopoiesis stimulating agents rhTPO Romiplostim Eltrombopag short TTR low sustained response rate N Engl J Med. 2007; 357(22):2237-47. Lancet. 2008;371(9610):395-403. Lancet. 2009;373(9664):641-8. N Engl J Med. 2010;363(20):1889-99. Int J Hematol. 2012;96(2):222-8. Blood. 2015;125(10):1541-7. Second-line treatment - 1

15 Rituximab long TTR high sustained response rate Blood. 2001;98(4):952-7. Ann Intern Med. 2007;146(1):25-33. Haematologica. 2008; 93(6):930-3. Blood. 2012;119(25):5989-95. Blood. 2015;125(10):1541-7. Lancet. 2015;385(9978):1653-61. Second-line treatment - 2

16 Splenectomy 32% sustained response surgical risk post-splenectomy state Blood. 2004;104(9):2623-34. Blood. 2013;121(23):4782-90. Second-line treatment - 3

17 DXM plus Rituximab DXM plus Eltrombopag rhTPO plus Rituximab DXM, Rituximab and Cyclosporin Blood. 2015;125(10):1541-7. Blood. 2010;115(14):2755-62. David GA. ASH Annual Meeting Abstracts, 2013 Blood. 2015 May 13. Epub ahead of print Combined therapy

18 Definition of response CR: platelet count > 100×10 9 /L and absence of bleeding R: platelet count > 30×10 9 /L and at least 2-fold increase the baseline count and absence of bleeding NR: platelet count < 30×10 9 /L or less than 2-fold increase of baseline

19 ITP: The Next Decade Restoration of immune tolerance Tregs/Bregs BAFF/BAFF-R antagonist MSC/Thalidomide MDSC DANA/Oseltamivir Anti-inflammatory macrophages/dasatinib

20 Thank you


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