Background Although loss of splenic function is the expected natural course for individuals with sickle cell disease (SCD), factors such as high HbF and coexistence of alpha thalassemia may ameliorate this process. We evaluated factors associated with two surrogate markers of spleen dysfunction, namely Howell–Jolly bodies (HJBs) and argyrophilic inclusion (AI) red cell counts, among patients with SCD. Methods Cross-sectional data of 182 patients with SCD (median age 11 y; 1–45 y) and 102 normal controls (median age 12 y; 1–32 y) were evaluated. Blood tests including full blood count, serum chemistry and high-performance liquid chromatography were performed. The HJB and AI red cell counts were performed on peripheral blood smears. Results The percentages of HJB and AI red cells rose significantly with increasing age in the SCD group. On regression analysis, the frequency of HJB red cells associated positively with mean corpuscular hemoglobin (MCH) (β=0.289; p=0.001) and negatively with HbF (β=−0.259; p=0.002). The AI red cell counts also associated positively with MCH (β=0.321; P=0.001) and negatively with HbF (β=−0.242; p=0.020). Conclusions Data from this study indicate that the negative association of HbF with both markers of splenic dysfunction among our patients with SCD residing in a malaria endemic region is similar to findings elsewhere of its ameliorating effect on splenic dysfunction.

中文翻译：

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疟疾流行地区镰状细胞病患者脾功能障碍相关的临床和实验室因素

背景 虽然脾功能丧失是镰状细胞病 (SCD) 患者的预期自然过程，但高 HbF 和 α 地中海贫血共存等因素可能会改善这一过程。我们评估了 SCD 患者中与脾功能障碍的两个替代标志物相关的因素，即豪威尔-乔利小体 (HJB) 和嗜银包涵体 (AI) 红细胞计数。方法 对 182 名 SCD 患者（中位年龄 11 岁；1-45 岁）和 102 名正常对照（中位年龄 12 岁；1-32 岁）的横断面数据进行评估。进行了血液检查，包括全血细胞计数、血清化学和高效液相色谱。对外周血涂片进行 HJB 和 AI 红细胞计数。结果 SCD组中HJB和AI红细胞的百分比随着年龄的增加而显着上升。回归分析显示，HJB 红细胞的频率与平均红细胞血红蛋白 (MCH) 呈正相关（β=0.289；p=0.001），与 HbF 呈负相关（β=-0.259；p=0.002）。AI 红细胞计数还与 MCH 呈正相关（β=0.321；P=0.001），与 HbF 呈负相关（β=-0.242；p=0.020）。结论 这项研究的数据表明，在我们居住在疟疾流行地区的 SCD 患者中，HbF 与脾功能障碍的两种标志物呈负相关，这与其他地方的研究结果相似，即 HbF 对脾功能障碍的改善作用。