Background Pneumococcal infections remain the main cause of overwhelming post-splenectomy infections, and purpura fulminans may develop in almost 20% of patients with overwhelming post-splenectomy infection. We aimed at describing the impact of asplenia/hyposplenia on the clinical features and the outcomes of adult patients admitted to the intensive care unit (ICU) for pneumococcal purpura fulminans. Methods A 17-year national multicenter retrospective cohort study included adult patients admitted to 55 French ICUs for an infectious purpura fulminans from 2000 to 2016. Patients with pneumococcal purpura fulminans were analyzed according to the absence or presence of asplenia/hyposplenia. Results Among the 306 patients admitted to the ICU for purpura fulminans, 67 (22%) had a pneumococcal purpura fulminans, of whom 34 (51%) had asplenia ( n = 29/34, 85%) or hyposplenia ( n = 5/34, 15%) and 33 (49%) had eusplenia. The prevalence of pneumococcal purpura fulminans was seven times higher in asplenic/hyposplenic patients compared to eusplenic patients with purpura fulminans ( n = 34/39, 87% vs. n = 33/267, 12%; p < 0.001). The median time interval between the occurrence of asplenia/hyposplenia and ICU admission was 20 [9–32] years. Pneumococcal vaccine coverage was 35% in asplenic/hyposplenic patients. Purpura was more frequently reported before ICU admission in asplenic/hyposplenic patients ( n = 25/34, 73% vs. n = 13/33, 39%; p = 0.01). The rate of bacteremia did not differ between asplenic/hyposplenic and eusplenic patients ( n = 31/34, 91% vs n = 27/33, 82%; p = 0.261). SAPS II (60 ± 14 vs. 60 ± 18; p = 0.244) and SOFA (13 [1–5] vs. 14 [1–4, 6]; p = 0.48) scores did not differ between asplenic/hyposplenic and eusplenic patients. There were no significant differences between asplenic/hyposplenic and eusplenic patients regarding the rate of limb amputation ( n = 9/34, 26% vs. 15/33, 45%; p = 0.11) and hospital mortality ( n = 20/34, 59% vs. n = 15/33, 45%; p = 0.27). Conclusions Half of pneumococcal purpura fulminans episodes occurred in asplenic or hyposplenic patients. Pneumococcal vaccine coverage was reported in one third of asplenic/hyposplenic patients. Half of pneumococcal purpura fulminans episodes occurred more than 20 years after splenectomy. Outcomes of pneumococcal purpura fulminans did not show significant differences between patients with or without asplenia or hyposplenia, although the small number of patients included limited our power to detect potential differences between groups.

中文翻译：

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无脾或脾功能减退患者的暴发性肺炎球菌性紫癜：法国多中心暴露-未暴露回顾性队列研究

背景 肺炎球菌感染仍然是脾切除术后感染的主要原因，几乎 20% 的脾切除术后感染患者可能发生暴发性紫癜。我们旨在描述无脾/脾虚低对因肺炎球菌性紫癜住进重症监护病房 (ICU) 的成年患者的临床特征和结果的影响。方法 一项为期 17 年的全国多中心回顾性队列研究纳入了 2000 年至 2016 年因感染性暴发性紫癜在法国 55 间重症监护病房收治的成年患者。根据是否存在无脾/脾功能减退对爆发性肺炎球菌性紫癜患者进行分析。结果 306 例因暴发性紫癜住进 ICU 的患者中，67 例（22%）有肺炎球菌性紫癜，其中 34 例（51%）有无脾（n = 29/34，85%) 或脾虚 (n = 5/34, 15%) 和 33 (49%) 有脾虚。与患有暴发性紫癜的正常脾脏患者相比，无脾/脾功能减退患者的肺炎球菌暴发性紫癜患病率高出 7 倍（n = 34/39, 87% vs. n = 33/267, 12%；p < 0.001）。发生无脾/脾虚低与入住 ICU 之间的中位时间间隔为 20 [9-32] 年。无脾/脾虚患者的肺炎球菌疫苗覆盖率为 35%。无脾/脾功能减退的患者在入住 ICU 前更常报告紫癜（n = 25/34，73% vs. n = 13/33，39%；p = 0.01）。无脾/低脾和正常脾患者的菌血症率没有差异（n = 31/34，91% vs n = 27/33，82%；p = 0.261）。SAPS II (60 ± 14 vs. 60 ± 18; p = 0.244) 和 SOFA (13 [1–5] vs. 14 [1–4, 6]; p = 0. 48) 评分在无脾/脾功能减退和正常患者之间没有差异。在肢体截肢率（n = 9/34，26% vs. 15/33，45%；p = 0.11）和住院死亡率（n = 20/34， 59% vs. n = 15/33, 45%；p = 0.27)。结论 一半的暴发性肺炎球菌性紫癜发作发生在无脾或脾虚的患者中。据报道，三分之一的脾脏/脾脏不足患者接种了肺炎球菌疫苗。一半的暴发性肺炎球菌性紫癜发作发生在脾切除术后 20 多年。暴发性肺炎球菌性紫癜的结果在有无无脾或脾虚的患者之间没有显着差异，