<p>Immune thrombocytopenia (ITP) is a heterogeneous disorder with fluctuating platelet&#xa0;counts. Current classifications do not adequately capture acute exacerbations in stable&#xa0;chronic ITP patients. We describe two chronic ITP patients with long-term stable&#xa0;platelet counts who experienced sudden, symptomatic thrombocytopenic flares. Both&#xa0;were managed with short courses of corticosteroids or IVIg while continuing prior&#xa0;therapy. Outcomes were assessed clinically and hematologically over extended followup.&#xa0;Both patients showed rapid and sustained responses, returning to baseline platelet&#xa0;counts without long-term therapy escalation. These episodes were distinguishable from&#xa0;refractory ITP by their identifiable triggers, episodic course, and excellent response to&#xa0;short-term treatment. We propose diagnostic criteria, key differentiators from refractory&#xa0;ITP, and a management algorithm. Acute on Chronic ITP is a distinct clinical entity&#xa0;characterized by sudden platelet declines in stable ITP patients, responsive to shortterm&#xa0;therapy. Recognizing this phenotype may prevent overtreatment, reduce&#xa0;healthcare costs, and improve patient outcomes. Formal incorporation into future ITP&#xa0;guidelines is warranted.</p>

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Flare-Ups in Chronic ITP: A Proposal for a Clinical Sub-Phenotype “Acute-on-Chronic ITP”

  • Sarthak Wadhera,
  • Arihant Jain,
  • Rudra Narayan Swain,
  • Debadrita Ray,
  • Prashant Sharma,
  • Pankaj Malhotra

摘要

Immune thrombocytopenia (ITP) is a heterogeneous disorder with fluctuating platelet counts. Current classifications do not adequately capture acute exacerbations in stable chronic ITP patients. We describe two chronic ITP patients with long-term stable platelet counts who experienced sudden, symptomatic thrombocytopenic flares. Both were managed with short courses of corticosteroids or IVIg while continuing prior therapy. Outcomes were assessed clinically and hematologically over extended followup. Both patients showed rapid and sustained responses, returning to baseline platelet counts without long-term therapy escalation. These episodes were distinguishable from refractory ITP by their identifiable triggers, episodic course, and excellent response to short-term treatment. We propose diagnostic criteria, key differentiators from refractory ITP, and a management algorithm. Acute on Chronic ITP is a distinct clinical entity characterized by sudden platelet declines in stable ITP patients, responsive to shortterm therapy. Recognizing this phenotype may prevent overtreatment, reduce healthcare costs, and improve patient outcomes. Formal incorporation into future ITP guidelines is warranted.