Male C, Koren D, Eichelberger B, Kaufmann K, Panzer S
Department for Pediatrics, Medical University Vienna, Austria.
Vox Sang. 2006 Aug;91(2):174-7. doi: 10.1111/j.1423-0410.2006.00797.x.
Patients with Glanzmann thrombasthenia (GT) may form isoantibodies which induce refractoriness or inhibition of function of transfused platelets. We monitored the survival and function of transfused platelets by flow cytometry and thrombelastography in a patient with GT. Gating on CD42a+ allowed identification of even a few transfused platelets. Only by gating on these CD41+ CD42a+ cells were we able to demonstrate their capability to bind fibrinogen and PAC-1 upon activation. Platelets were rapidly cleared from the circulation as a result of boosted isoantibodies. The contribution of transfused platelets to clot formation was also demonstrated by thrombelastography by blocking their function with abciximab.
患有Glanzmann血小板无力症(GT)的患者可能会形成同种抗体,从而导致输注的血小板出现难治性或功能抑制。我们通过流式细胞术和血栓弹力图监测了一名GT患者输注血小板的存活情况和功能。通过对CD42a+进行门控,即使是少量输注的血小板也能被识别出来。只有通过对这些CD41+ CD42a+细胞进行门控,我们才能证明它们在激活后结合纤维蛋白原和PAC-1的能力。由于同种抗体增强,血小板从循环中迅速清除。通过使用阿昔单抗阻断输注血小板的功能,血栓弹力图也证明了其对血栓形成的贡献。