Primary immune thrombocytopenia in adults. Guidelines for diagnosis and treatment anno 2013 proposed by the Belgian Hematological Society

The Belgian Hematological Society (BHS) guideline panel on adult primary immune thrombocytopenia (ITP) reviewed the recent literature on diagnosis and treatment to make recommendations on the best strategies for frontline and subsequent-line treatment. No treatment is necessary for patients with platelet counts higher than 30000/μl in the absence of bleeding symptoms. Patients newly diagnosed or relapsing after a long-term treatment-free period can be managed with corticosteroids with or without intravenous immunoglobulins. A second line therapy is indicated for those patients who are intolerant or unresponsive to or relapse after initial corticosteroid treatment and have a risk of bleeding. The guideline panel recommends splenectomy as it is the treatment with the highest curative potential and an acceptable safety profile. If possible, splenectomy should be delayed to at least twelve months after diagnosis as spontaneous remission can occur in this time period. Thrombopoietin receptor (TPO-R) agonists are recommended for patients who are refractory to or relapse after splenectomy or who have a contra-indication to splenectomy irrespective of the duration of ITP. The guideline panel agrees that rituximab, azathioprine, cyclophosphamide, cyclosporine A, danazol, dapsone, mycophenolate mofetil and vincristine/vinblastine are potential treatment options, especially for patients refractory to TPO-R agonists