血小板减少性紫癜 Thrombocytopenic purpura.ppt

* Platelet transfusions may produce some increase in platelet numbers in many patients, often diminish bleeding for a time.They should be reserved for such life-threatening emergencies or for the immediate preoperative treatment of patients with serious hemorrhage before splenectomy. In patients with platelet counts above 50×109/L, preoperative platelet transfusions are not indicated. Platelet replacement should be avoided in patients with chronic ITP because of the possible development of alloantibodies. * High-dose immunoglobulin. The mechanism of this therapeutic effect is not entirely clear, but most evidence points to blockade of the Fc receptors of the reticuloendothelial cells, and perhaps neutralization of antiplatelet autoantibodies by antiidiotypic antibodies in the preparations. A regimen of 400mg/kg/day for 5 days usually is recommended. Among patients with cITP, platelet counts increased in about 75% of patients and reached normal levels in about half of patients * A number of models have been proposed to explain the efficacy of IVIgG in ITP (221-226). The two most widely accepted explanations are impairment of phagocytic cell function and suppression of autoantibody synthesis. Most clinicians think that IVIgG works by reticuloendothelial cell blockade (medical splenectomy) (Fig. 32-4). The usual total dose of IVIgG (2 g/kg) raises the serum IgG concentration by twofold to fourfold, a level that has been shown to block the clearance of radiolabeled, IgG-sensitized RBCs (227,228). Consistent with this view is the observation that disease-related polyclonal hypergammaglobulinemia also is associated with impaired reticuloendothelial cell function (229). We believe that the monomeric IgG is in equilibrium with the Fc receptor on the phagocytic cells. Even though the binding affinity of Fc receptors for monomeric IgG is low, the high plasma concentration of IgG results in these receptors being occupied (229). It is possible that small quantities of contam