Valentine Brousse, Sara El Hoss, Pierre Isnard, Sandrine Laurance, Camille Lambert, Liza Ali, Arnaud Bonnard, Carmen Capito, Sabine Sarnacki, D. Berrebi, Berengère Koehl, Malika Benkerrou, Florence Missud, Laurent Holvoet, Ghislaine Ithier, Mariane de Montalembert, Slimane Allali, Leon Tshilolo, Jacques Diebold, Thierry Jo Molina
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An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy–Gamna, and RBC trapping) combined into a severity score was established, as well as B-cell follicles analysis. Quantification on digitalized slides of iron deposition, RBC trapping, and red pulp fibrosis was additionally performed. Spleens from 22 children with SCA, eight with HS, eight with TDT, and three healthy controls (HC) were analyzed. Median age at splenectomy was not different between SCA and HS patients, 6.05 years (range: 4.5–16.0) versus 4.75 (range: 2.2–9.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions<i>.</i> Contrary to previous reports, B-cell follicles were generally preserved in SCA. While RBC trapping was significantly increased in both SCA and HS (compared to TDT and HC), quantitative fibrosis and overall red pulp severity score were significantly increased in SCA spleens compared to other conditions. Moreover, there was an inverse correlation between quantitative fibrosis and number of B-cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B-cell subpopulations defects.</p>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":10.1000,"publicationDate":"2024-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.27374","citationCount":"0","resultStr":"{\"title\":\"Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia\",\"authors\":\"Valentine Brousse, Sara El Hoss, Pierre Isnard, Sandrine Laurance, Camille Lambert, Liza Ali, Arnaud Bonnard, Carmen Capito, Sabine Sarnacki, D. 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引用次数: 0
摘要
镰状细胞性贫血(SCA)和遗传性球形红细胞增多症(HS)的共同特点是红细胞(RBC)周转增加导致脾脏红细胞吞噬功能增强,而SCA的特殊性在于易感染。本研究分析了 SCA 儿童患者脾脏的组织学病变,这些病变与既往临床病史密切相关,并与 HS、健康和输血的β地中海贫血患者(TDT)进行了比较。对红髓基本病变(红髓纤维化、铁沉积、甘迪-甘纳数量和红细胞捕获)进行评估,并将其合并为严重程度评分,同时还进行了 B 细胞滤泡分析。此外,还对数字化切片上的铁沉积、RBC潴留和红髓纤维化进行了量化。对 22 名 SCA 患儿、8 名 HS 患儿、8 名 TDT 患儿和 3 名健康对照组(HC)的脾脏进行了分析。SCA和HS患者切除脾脏时的中位年龄没有差异,分别为6.05岁(范围:4.5-16.0)和4.75岁(范围:2.2-9.5)。与其他情况相比,SCA 脾脏中发现了明显的异质性。与之前的报告相反,B细胞滤泡在SCA中普遍保留。与TDT和HC相比,SCA和HS的红细胞捕获均明显增加,但与其他情况相比,SCA脾脏的定量纤维化和总体红髓严重程度评分明显增加。此外,定量纤维化与 B 细胞滤泡数量之间存在反相关性,这可能是通过红髓巨噬细胞清除能力受损和 B 细胞亚群缺陷,将这两个部分以及脾脏纤维化与 SCA 的感染易感性联系起来。
Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia
While sickle cell anemia (SCA) and hereditary spherocytosis (HS) share common features of increased spleen erythrophagocytosis due to increased red blood cell (RBC) turnover, SCA is specifically characterized by susceptibility to infections. In this study, histological lesions in the spleens of pediatric patients with SCA were analyzed, in close correlation with past clinical history and comparatively to HS, healthy and transfused β-thalassemia patients (TDT). An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy–Gamna, and RBC trapping) combined into a severity score was established, as well as B-cell follicles analysis. Quantification on digitalized slides of iron deposition, RBC trapping, and red pulp fibrosis was additionally performed. Spleens from 22 children with SCA, eight with HS, eight with TDT, and three healthy controls (HC) were analyzed. Median age at splenectomy was not different between SCA and HS patients, 6.05 years (range: 4.5–16.0) versus 4.75 (range: 2.2–9.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions. Contrary to previous reports, B-cell follicles were generally preserved in SCA. While RBC trapping was significantly increased in both SCA and HS (compared to TDT and HC), quantitative fibrosis and overall red pulp severity score were significantly increased in SCA spleens compared to other conditions. Moreover, there was an inverse correlation between quantitative fibrosis and number of B-cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B-cell subpopulations defects.
期刊介绍:
The American Journal of Hematology offers extensive coverage of experimental and clinical aspects of blood diseases in humans and animal models. The journal publishes original contributions in both non-malignant and malignant hematological diseases, encompassing clinical and basic studies in areas such as hemostasis, thrombosis, immunology, blood banking, and stem cell biology. Clinical translational reports highlighting innovative therapeutic approaches for the diagnosis and treatment of hematological diseases are actively encouraged.The American Journal of Hematology features regular original laboratory and clinical research articles, brief research reports, critical reviews, images in hematology, as well as letters and correspondence.