Lum L G, Tubergen D G, Corash L, Blaese R M
N Engl J Med. 1980 Apr 17;302(16):892-6. doi: 10.1056/NEJM198004173021604.
The Wiskott-Aldrich syndrome is an X-linked immunodeficiency disorder consisting of the triad of frequent infections, eczema, and profound thrombocytopenia. We evaluated the effects of splenectomy on hemostatic improvement and subsequent clinical course in 16 patients with the Wiskott-Aldrich syndrome. All 16 had an increase in platelet counts to at least 100,000 per cubic millimeter after splenectomy, with the mean increasing from 19,900 per cubic millimeter preoperatively to 262,700 per cubic millimeter after splenectomy. In addition, platelet size, which is characteristically small in this disease, also became normal. Survival after splenectomy correlated with the prophylactic use of antibiotics. Five of seven patients not taking prophylactic antibiotics died of sepis within 33 months of surgery. The mean survival of the nine patients maintained with prophylactic antibiotics, however, was at least 91.4 months, with six of these patients still alive an average of 11.0 years or more after splenectomy. Thus, splenectomy is a useful therapy for a major cause of morbidity and mortality in this complex syndrome.
威斯科特-奥尔德里奇综合征是一种X连锁免疫缺陷疾病，其特征为频繁感染、湿疹和严重血小板减少三联征。我们评估了脾切除术对16例威斯科特-奥尔德里奇综合征患者止血改善及后续临床病程的影响。所有16例患者脾切除术后血小板计数均增加至至少每立方毫米100,000，平均术前每立方毫米19,900增加至脾切除术后每立方毫米262,700。此外，该病特征性的小血小板大小也恢复正常。脾切除术后的生存与预防性使用抗生素相关。7例未使用预防性抗生素的患者中有5例在术后33个月内死于败血症。然而，9例接受预防性抗生素治疗患者的平均生存期至少为91.4个月，其中6例患者在脾切除术后平均存活11.0年或更长时间。因此，脾切除术是治疗这一复杂综合征中发病和死亡主要原因的有效疗法。
