Corash L, Shafer B, Blaese R M
Blood. 1985 Jun;65(6):1439-43.
Wiskott-Aldrich syndrome (WAS) thrombocytopenia is frequently improved by splenectomy, although the mechanism of the thrombocytopenia and its resolution are unknown. Previous studies in two patients have shown that mean platelet volume, which is characteristically reduced in WAS, increased along with platelet count postsplenectomy. Additional studies in a limited number of patients have also demonstrated that platelet-associated immunoglobulin G (PAIgG) is elevated presplenectomy, but to date no postsplenectomy data have been reported. The present study was performed to more fully evaluate the effect of splenectomy on platelet volume and PAIgG in WAS. Before splenectomy, mean platelet volume was reduced but platelet size was broadly distributed with substantial overlap of the normal range. PAIgG was significantly elevated in 13 of 14 presplenectomy WAS patients (means = 78.9 fg per platelet) and fell to normal levels postoperatively (means = 4.0 fg per platelet). Platelet count and clinical status improved postsplenectomy, and mean platelet volume and platelet volume distribution returned to the normal range. WAS subjects who relapsed with recurrent thrombocytopenia redeveloped elevated PAIgG but maintained normal platelet size. The spleen appears to play a critical role in a process that may be immunologically mediated and results in reduced platelet size.
威斯科特-奥尔德里奇综合征（WAS）患者的血小板减少症常可通过脾切除术得到改善，尽管血小板减少症的机制及其缓解机制尚不清楚。此前对两名患者的研究表明，WAS患者特征性降低的平均血小板体积在脾切除术后随血小板计数增加而升高。对少数患者的进一步研究还表明，脾切除术前血小板相关免疫球蛋白G（PAIgG）升高，但迄今为止尚未有脾切除术后的数据报道。本研究旨在更全面地评估脾切除术对WAS患者血小板体积和PAIgG的影响。脾切除术前，平均血小板体积降低，但血小板大小分布广泛，与正常范围有大量重叠。14例脾切除术前的WAS患者中有13例PAIgG显著升高（平均每血小板78.9 fg），术后降至正常水平（平均每血小板4.0 fg）。脾切除术后血小板计数和临床状况改善，平均血小板体积和血小板体积分布恢复到正常范围。复发血小板减少症的WAS患者重新出现PAIgG升高，但血小板大小维持正常。脾脏似乎在一个可能由免疫介导且导致血小板大小减小的过程中起关键作用。
