Hebbar M, Kaplan C, Caulier M T, Wattel E, Morel P, Wetterwald M, Bauters F, Fenaux P
Department of Internal Medicine, CHU Lille, France.
Br J Haematol. 1996 Jul;94(1):112-5. doi: 10.1046/j.1365-2141.1996.6322262.x.
We prospectively studied the mechanism of thrombocytopenia in 30 patients with myelodysplastic syndrome (MDS), who had a platelet count <or= 70 x 10(9)/l, and marrows blasts <or= 10% by analysis of platelet lifespan, platelet-bound IgG antibodies by platelet radioactive antiglobulin test (PRAT), and circulating and platelet-bound autoantibodies specifically directed against platelet glycoproteins by indirect and direct MAIPA test. PRAT analysis was positive in 21/30 patients (70%), but antiplatelet antibodies were found in only eight by the MAIPA test. Platelet lifespan was shortened in five cases, only one of whom had a positive MAIPA test. Patients were treated by danazol (600 mg/d), and 9/26 evaluable patients (35%) responded, including three of the five patients with shortened platelet lifespan. No correlation between platelet lifespan, results of PRAT and MAIPA tests, and response to danazol was found. In conclusion, specific antiplatelet autoantibodies, reduced platelet lifespan, and efficacy of androgen therapy on platelets were seen in 20%, 17% and 35% of MDS with thrombocytopenia, respectively, but no correlation between these findings was found in this study.
我们前瞻性地研究了30例骨髓增生异常综合征(MDS)患者血小板减少的机制,这些患者血小板计数≤70×10⁹/L,骨髓原始细胞≤10%,通过分析血小板寿命、血小板放射免疫球蛋白试验(PRAT)检测血小板结合的IgG抗体,以及通过间接和直接单克隆抗体特异性血小板抗原固定试验(MAIPA)检测针对血小板糖蛋白的循环和血小板结合自身抗体。PRAT分析在21/30例患者(70%)中呈阳性,但通过MAIPA试验仅在8例患者中发现抗血小板抗体。5例患者血小板寿命缩短,其中仅1例MAIPA试验呈阳性。患者接受达那唑(600mg/d)治疗,26例可评估患者中有9例(35%)有反应,包括5例血小板寿命缩短患者中的3例。未发现血小板寿命、PRAT和MAIPA试验结果与达那唑反应之间存在相关性。总之,在20%、17%和35%的血小板减少的MDS患者中分别观察到特异性抗血小板自身抗体、血小板寿命缩短和雄激素治疗对血小板的疗效,但本研究未发现这些结果之间存在相关性。