Pneumococcal purpura fulminans in asplenic or hyposplenic patients: a French multicenter exposed-unexposed retrospective cohort study

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.