Zanella A, Barosi G, Berzuini A, Colombo M B, Quaglini S, Volpes D, Sirchia G
Centro Trasfusionale e di Immunologia dei Trapianti, Ospedale Maggiore Policlinico, Milano, Italy.
Am J Hematol. 1989 Jun;31(2):81-6. doi: 10.1002/ajh.2830310202.
Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, transferrin saturation, and serum ferritin than normal matched controls and normal zinc protoporphyrin (ZnPP) levels. On the contrary, nonsplenectomized patients presenting with mild to severe anemia had higher red cell ZnPP concentrations than both splenectomized subjects and matched normal controls. ZnPP in nonsplenectomized patients correlated inversely with Hb concentration, mean corpuscular volume (MCV), mean red cell hemoglobin concentration (MCHC), transferrin saturation, and serum iron, and directly with reticulocyte count. At multiple regression analysis only Hb concentration was a significant explanatory variable for high ZnPP. The authors conclude that a number of nonsplenectomized HS patients have relative iron deficiency primarily because of expansion of erythropoiesis caused by anemia.
对73例遗传性球形红细胞增多症（HS）患者（58例未行脾切除术，15例已行脾切除术）进行研究，以评估铁状态及红细胞生成中铁供应的充足性。已行脾切除术的患者血红蛋白水平处于正常或正常上限范围，其血清铁、转铁蛋白饱和度和血清铁蛋白水平高于正常匹配对照组，且锌原卟啉（ZnPP）水平正常。相反，患有轻至重度贫血的未行脾切除术的患者，其红细胞ZnPP浓度高于已行脾切除术的患者及匹配的正常对照组。未行脾切除术患者的ZnPP与血红蛋白浓度、平均红细胞体积（MCV）、平均红细胞血红蛋白浓度（MCHC）、转铁蛋白饱和度和血清铁呈负相关，与网织红细胞计数呈正相关。在多元回归分析中，只有血红蛋白浓度是高ZnPP的显著解释变量。作者得出结论，许多未行脾切除术的HS患者存在相对铁缺乏，主要是由于贫血导致红细胞生成增加。
