Boruchov Donna M, Gururangan Sri, Driscoll M Catherine, Bussel James B
The Brookdale University Hospital and Medical Center Department of Pediatrics, Hematology Oncology, Brooklyn, NY, USA.
Blood. 2007 Nov 15;110(10):3526-31. doi: 10.1182/blood-2007-01-065763. Epub 2007 Aug 13.
Patients with severe immune thrombocytopenic purpura (ITP) may require an acute increase in the platelet count for surgery or ongoing hemorrhage as well as long-term maintenance treatment. Certain of these patients may be refractory to steroids, intravenous anti-D, intravenous immunoglobulin (IVIG), and splenectomy. Therefore, acute platelet increases were studied in 35 patients completely unresponsive to IVIG or high-dose steroid treatment. Because of their lack of response to either or both single agents, these patients were administered a 3- or 4-drug combination including IVIG 1 g/kg, intravenous methylprednisolone 30 mg/kg, Vinca alkaloids (VCR 0.03 mg/kg), and/or intravenous anti-D (50-75 microg/kg). Subsequent maintenance therapy with the oral combination of danazol (10-15 mg/kg) and azathioprine (2 mg/kg) was given to 18 of the 35 patients. Seventy-one percent of the patients responded to the intravenous combination treatment with acute platelet increases of at least 20 x 10(9)/L to a level greater than 30 x 10(9)/L. Two thirds of the patients given maintenance therapy achieved stable platelet counts greater than 50 x 10(9)/L without other treatments. One patient developed an ileus, but otherwise there was little toxicity of combination treatment. Combination chemotherapy is a useful approach for patients with ITP refractory to conventional treatments both for acute induction and for long-term maintenance therapy.
重度免疫性血小板减少性紫癜（ITP）患者在手术或持续出血时可能需要迅速提高血小板计数，同时也需要长期维持治疗。其中一些患者可能对类固醇、静脉注射抗-D、静脉注射免疫球蛋白（IVIG）和脾切除术无效。因此，对35例对IVIG或高剂量类固醇治疗完全无反应的患者进行了急性血小板增加的研究。由于这些患者对单一药物或两种药物均无反应，因此给予他们一种包含IVIG 1 g/kg、静脉注射甲泼尼龙30 mg/kg、长春花生物碱（长春新碱0.03 mg/kg）和/或静脉注射抗-D（50 - 75 μg/kg）的三联或四联药物组合。35例患者中有18例随后接受了达那唑（10 - 15 mg/kg）和硫唑嘌呤（2 mg/kg）口服联合的维持治疗。71%的患者对静脉联合治疗有反应，急性血小板增加至少20×10⁹/L，达到大于30×10⁹/L的水平。接受维持治疗的患者中有三分之二在未进行其他治疗的情况下血小板计数稳定在大于50×10⁹/L。有1例患者发生肠梗阻，但联合治疗的毒性总体较小。联合化疗对于常规治疗无效的ITP患者来说，无论是急性诱导还是长期维持治疗都是一种有效的方法。
