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REFRACTORY ITP

REFRACTORY ITP. Persistence of thrombocytopenia after initial therapy, including splenectomy, with the need of active treatment to maintain a safe platelet count or to avoid bleeding symptoms. RESPONSE TO TREATMENT IN ITP: SUMMARY OF AN IDEAL 10-YRS FOLLOW UP.

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REFRACTORY ITP

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  1. REFRACTORY ITP Persistence of thrombocytopenia after initial therapy, including splenectomy, with the need of active treatment to maintain a safe platelet count or to avoid bleeding symptoms

  2. RESPONSE TO TREATMENT IN ITP: SUMMARY OF AN IDEAL 10-YRS FOLLOW UP • Moderate ITP: no treatment; remain stable; 5-10 % will be reclassified as secondary  • Severe ITP: • < 5 % primarily refractory to first line therapies • 10–20 % stable response to corticosteroids • 80-90 % require splenectomy within 6 months: • 5 - 10 % late spontaneous remission • 65 – 75 % stable response • 15 – 20 % require second line treatment (at least on demand): • < 5% spontaneous remission • 75-85 % responsive (10-15% of initial cohort) • 10-15% unresponsive (~5% of initial cohort)

  3. Refractory ITP Hemorrhagic risk of ITP <30.000/mcL (Cohen et al, 2000) Fatal hemorrhage -meta-analysis of 17 studies, 1.817 patients with ‘platelet count < 30.000/mcL -mean follow-up: from 1.258 to 3.023 patient-year -49 cases (31 SNC, 8 others sites) (9 studies) -annual rate : from 0.0162 to 0.0389 cases per patient-year -rate for age: 0.004 cases per patient-year for age < 40 years 0.13 cases per patient-years for age > 60 years

  4. Refractory ITP Hemorrhagic risk of ITP <30.000/mcL (Cohen et al, 2000) Non-fatal hemorrhage -meta-analysis of 17 studies, 1.817 patients with ‘platelet count < 30.000/mcL -mean follow-up: from 77 to 105 patient-years -29 cases (major episodes) (2 studies) -annual rate : from 0.276 a 0.376 cases per patient-year -rate for age: 0.03cases per patient-year for age < 40 years 0.71 cases per patient-year for age > 60 years

  5. Refractory ITPmortality risk of ITP <30.000/mcL (Portijelie et al, 2000) • 4 deaths within the first 2 years follow-up (1 for hemorrhage, 2 for infection during steroid therapy, 1 for post-splenectomy sepsis) - 20 deaths during the long term follow-up(median: 10 years): 2 ITP- related (1 for hemorrhage, 1 for infection) 18 ITP not related

  6. Refractory ITP Mortality in refractory patients Author Haemorrhage-related deaths Deaths due to infection Shatner et al 1994 1/120-480 (0.8%) 2/120-480 (1.6%)  George et al 1996 25/465 (5%) NR  Cohen et al 2000 49/1817 (2.6%) NR Vianelli et al 2001 1/33 (3%) 0/33 Portielje et al 2001 1/12 (8.3%) 1/12 (8.3%)  McMillan et al 2001 3/13 (23%) 2/13 (15%) Total 80/2460 (3.2%) 5/178 (2.8%)

  7. Refractory ITPa challenge Propose a widely accepted definition of refractory ITP Prospective inception cohort study (GIMEMA experience) for refractory ITP patients, to identify the clinical outcome Identify high risk patients for bleeding Clinical trial to identify splenectomy-sparing therapy Clinical and biological prospective study to identify factors associated with response to splenectomy Randomized clinical trial to identify the treatments with the most favourable cost-benefit ratio, including quality of life assessment

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