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目的:揭示遗传性球形红细胞增多症（HS）骨髓红系造血的代偿特征，探究不同程度贫血对骨髓造血代偿的影响。方法:收集2014年7月至2020年9月中国医学科学院血液病医院确诊的HS患者临床及实验室资料，以外周血网织红细胞绝对值作为替代参数，评估骨髓红系造血代偿能力，并对不同贫血程度HS患者红系造血代偿进行比较。结果:① 302例HS患者中代偿性溶血病（代偿组）115例，轻、中、重度贫血（失代偿组）患者分别为74、90、23例。②失代偿组血清红细胞生成素（EPO）水平与HGB呈负相关（n rs=-0.585，n P<0.001）。③ HS患者的中位网织红细胞计数（ARC）0.34（0.27，0.44）×10n 12/L，约为正常人的4.25倍，最大ARC 0.81×10n 12/L，约为正常人的10倍；代偿组的中位ARC 0.29（0.22，0.38）×10n 12/L，约为正常人的3.63倍，失代偿组中位ARC 0.38（0.30，0.46）×10n 12/L，明显高于代偿组（n z=4.999，n P=0.003），达正常人的4.75倍。④代偿组的ARC与HGB呈负相关（n r=-0.177，n P=0.002）；失代偿组ARC与HGB呈正相关（n rs=0.191，n P=0.009），轻、中、重度贫血组间ARC差异无统计学意义（n χ2=4.588，n P=0.101）。⑤轻、中、重度贫血组的中位未成熟网织红细胞指数（IRF）分别为13.1%（9.1%，18.4%）、17.0%（13.4%，20.8%）、17.8%（14.6%，21.8%），轻度贫血组IRF小于中度及重度贫血组（n Padj值均<0.05），而中度和重度贫血组间差异无统计学意义（n Padj=1.000）；轻、中、重度贫血组的中位新生网织红细胞计数分别为5.09（2.60，7.74）×10n 10/L、6.24（4.34，8.83）×10n 10/L、7.00（3.07，8.22）×10n 10/L，组间差异无统计学意义（n χ2=3.081，n P=0.214）。n 结论:HS骨髓红系造血代偿随不同程度红细胞减少而增加，维持HGB在正常水平；而一经贫血发生，造血代偿即已达最大水平，不因贫血加重、EPO增加而进一步代偿增加。“,”Objective:To reveal the compensatory features of bone marrow (BM) erythropoiesis in hereditary spherocytosis (HS) and to explore the effect of diferent hemoglobin levels on this compensation.Methods:Clinical and laboratory data of patients with HS were collected, and the peripheral blood absolute reticulocytes counts value was taken as the surrogate parameter to evaluate the ability of erythropoiesis compensation. BM erythropoiesis compensation in HS with diferent degrees of anemia were evaluated.Results:①Three hundred and two patients were enrolled, including 115 with compensated hemolytic disease, 74 with mild anemia, 90 with moderate anemia, and 23 with severe anemia. ②Hemoglobin (HGB) was negatively correlated with serum erythropoietin in the decompensated hemolytic anemia group (EPO; n rs=-0.585, n P<0.001) . ③The median absolute reticulocyte count (ARC) of HS patients was 0.34 (0.27, 0.44) ×10n 12/L, up to 4.25 times that of normal people. The maximum ARC was 0.81×10n 12/L, about 10 times that of normal people. The median ARC of patients with compensated hemolytic disease was 0.29 (0.22, 0.38) ×10n 12/L, up to 3.63 times that of normal people. The median ARC of patients with hemolytic anemia was 0.38 (0.30, 0.46) ×10n 12/L, which was significantly higher than the patients with compensated hemolytic disease, up to 4.75 times that of normal people (n z=4.999, n P=0.003) . ④ ARC was negatively correlated with HGB in the compensated hemolytic disease group (n rs=-0.177, n P=0.002) and positively correlated with HGB in the decompensated hemolytic anemia group (n rs=0.191, n P=0.009) . There was no significant difference in the ARC among patients with mild, moderate, and severe anemia (n χ2=4.588, n P=0.101) . ⑤The median immature reticulocyte production index of the mild, moderate, and severe anemia groups was 13.1% (9.1%, 18.4%) , 17.0% (13.4%, 20.8%) , and 17.8% (14.6%, 21.8%) , respectively; the mild anemia group had lower index values than the moderate and severe anemia groups (n Padj values were both<0.05) , but there was no significant difference between the latter groups (n Padj=1.000) . The median immature reticulocyte count of patients in the mild, moderate, and severe groups was 5.09 (2.60, 7.74) ×10n 10/L, 6.24 (4.34, 8.83) ×10n 10/L, and 7.00 (3.07, 8.22) ×10n 10/L, respectively; there was no significant difference among the groups (n χ2=3.081, n P=0.214) .n Conclusion:HGB can be maintained at a normal level through bone marrow erythropoiesis, while red blood cells are reduced in HS. However, once anemia develops, the bone marrow exerts its maximum erythropoiesis capacity and does not increase, regardless of anemia aggravation or serum EPO increase.