Department of Medicine, University of Connecticut, 263 Farmington Ave, Farmington, CT, 06030, USA.
Department of Hematology, Universidade de São Paulo, São Paulo, SP, Brazil.
Ann Hematol. 2022 Sep;101(9):1951-1957. doi: 10.1007/s00277-022-04918-4. Epub 2022 Jul 15.
Sickle cell disease (SCD) is associated with multiple known complications and increased mortality. This study aims to further understand the profile of intensive care unit (ICU) admissions of SCD patients. In this single-center retrospective cohort (approval number 0926-11), we evaluated SCD-related ICU admissions at our hospital in São Paulo, Brazil. Admissions were clustered using clinical data and organ dysfunction at ICU admission. A hierarchical clustering method was used to distinguish phenotypes. From 140 admissions obtained, 125 were included. The mean age was 30 years, 48% were male, and SS genotype was predominant (71.2%). Non-surgical causes of admissions accounted for 85.6% (n = 107). The mean Sequential Organ Failure Assessment score (SOFA) was 4 (IQR 2-7). Vasopressors were required by 12% and mechanical ventilation by 17.6%. After analysis of the average silhouette width, the optimal number of clusters was 3: cluster 1 (n = 69), cluster 2 (n = 25), cluster 3 (n = 31). Cluster 1 had a mean age of 29 years, 87% of SS genotype, and mean SOFA of 4. Cluster 2 had a mean age of 37 years, 80% of SS genotype, and mean SOFA of 8. Cluster 3 had a mean age of 26 years, 29% of SS genotype, and mean SOFA of 3. The need for mechanical ventilation was 11.6%, 44%, and 9.7%, respectively. Mortality was significantly higher in cluster 2 (44%, p = 0.012). This cohort of critical SCD admissions suggested the presence of three different profiles. This can be informative in the ICU setting to identify SCD patients at higher risk of worse outcomes.
镰状细胞病（SCD）与多种已知并发症和死亡率增加有关。本研究旨在进一步了解 SCD 患者入住重症监护病房（ICU）的情况。在这项单中心回顾性队列研究中（批准号 0926-11），我们评估了巴西圣保罗我院 SCD 相关 ICU 入院情况。入院时根据临床数据和器官功能障碍进行聚类。使用层次聚类方法来区分表型。从获得的 140 例入院中，纳入了 125 例。平均年龄为 30 岁，48%为男性，SS 基因型占主导地位（71.2%）。非手术原因导致的入院占 85.6%（n=107）。平均序贯器官衰竭评估评分（SOFA）为 4（IQR 2-7）。12%需要升压药，17.6%需要机械通气。分析平均轮廓宽度后，最佳聚类数为 3 个：第 1 组（n=69）、第 2 组（n=25）和第 3 组（n=31）。第 1 组的平均年龄为 29 岁，SS 基因型占 87%，SOFA 平均为 4。第 2 组的平均年龄为 37 岁，SS 基因型占 80%，SOFA 平均为 8。第 3 组的平均年龄为 26 岁，SS 基因型占 29%，SOFA 平均为 3。机械通气的需求分别为 11.6%、44%和 9.7%。第 2 组的死亡率明显更高（44%，p=0.012）。这组严重 SCD 入院患者提示存在三种不同的表型。这在 ICU 环境中可以提供信息，以识别 SCD 患者中预后较差的风险更高的患者。
