Arora Rahul Darshan, Dass Jasmita, Maydeo Seema, Arya Vandana, Kotwal Jyoti, Bhargava Manorama
a Department of Hematology , Sir Ganga Ram Hospital , New Delhi , India.
Hematology. 2018 Aug;23(7):413-416. doi: 10.1080/10245332.2018.1423879. Epub 2018 Jan 16.
Hereditary spherocytosis (HS) is the most common congenital hemolytic anemia, characterized by anemia, jaundice, and splenomegaly. The diagnosis of HS relies on symptoms of hemolysis, a family history of HS, and a positive laboratory test which is usually the osmotic fragility test (OFT). We conducted a study to assess the utility of mean corpuscular hemoglobin concentration (MCHC), mean corpuscular volume (MCV), mean sphered cell volume (MSCV), and mean reticulocyte volume (MRV) in the diagnosis of HS and if these are helpful in distinguishing cases of HS from immune hemolytic anemia.
A total of 102 patients suspected to have HS were enrolled. In addition 10 cases of immune hemolytic anemia (IHA) were included in the study and performance of the above screening tests was evaluated. The diagnosis of HS was based on incubated OFT, eosin 5'-maleimide (EMA) dye binding test, and flowcytometric OFT.
A total of 29 patients were diagnosed as having HS. The sensitivity and specificity for diagnosis HS by MCHC > 35 g/dL was 44.82%, and ΔMCV-MSCV > 10 fL has a sensitivity and specificity of 82.75% and 95.9% for diagnosis of HS. Using an algorithm of ΔMCV-MSCV > 10 fL and ΔMRV-MSCV < 25, for the differentiation of HS from IHA had sensitivity of 68.9% and specificity of 98.8%.
遗传性球形红细胞增多症（HS）是最常见的先天性溶血性贫血，其特征为贫血、黄疸和脾肿大。HS的诊断依赖于溶血症状、HS家族史以及实验室检查阳性结果，通常为渗透脆性试验（OFT）。我们开展了一项研究，以评估平均红细胞血红蛋白浓度（MCHC）、平均红细胞体积（MCV）、平均球形红细胞体积（MSCV）和平均网织红细胞体积（MRV）在HS诊断中的效用，以及这些指标是否有助于鉴别HS与免疫性溶血性贫血。
共纳入102例疑似患有HS的患者。此外，研究还纳入了10例免疫性溶血性贫血（IHA）患者，并评估了上述筛查试验的表现。HS的诊断基于孵育后的OFT、嗜酸性5'-马来酰亚胺（EMA）染料结合试验和流式细胞术OFT。
共有29例患者被诊断为HS。MCHC＞35 g/dL诊断HS的敏感性和特异性分别为44.82%，而ΔMCV-MSCV＞10 fL诊断HS的敏感性和特异性分别为82.75%和95.9%。使用ΔMCV-MSCV＞10 fL和ΔMRV-MSCV＜25的算法来鉴别HS与IHA，敏感性为68.9%，特异性为98.8%。
