Murphy P T, Quinn J P, O'Donghaile D, Swords R, O'Donnell J R
Department of Haematology, Beaumont Hospital, Dublin 9, Ireland.
Ann Hematol. 2006 Jul;85(7):455-7. doi: 10.1007/s00277-006-0107-3. Epub 2006 Mar 28.
Raised percentage hypochromic red cells (%HRC) were detected at diagnosis in 10 of 34 consecutive patients with low-risk myelodysplastic syndrome (MDS) [refractory anemia (RA) (4/26) and RA with ring sideroblasts (6/8)], all of whom had normal or increased serum ferritin and bone marrow iron stores. Elevated %HRC has persisted in all 10 cases and subsequently developed in another RA patient who later had a complete remission of MDS with normalisation of %HRC after a respiratory tract infection. A strong positive correlation was found between %HRC and erythrocyte zinc protoporphyrin levels in 11 MDS patients tested (p=0.01), suggesting that functional iron deficiency contributes to ineffective erythropoiesis in cases of MDS with raised %HRC. Five of seven patients with elevated %HRC had satisfactory haemoglobin responses to a trial of human recombinant erythropoietin without iron supplementation.
在34例连续的低危骨髓增生异常综合征(MDS)患者[难治性贫血(RA)(26例中的4例)和伴有环形铁粒幼细胞的RA(8例中的6例)]中,10例在诊断时检测到低色素红细胞百分比(%HRC)升高,所有这些患者的血清铁蛋白和骨髓铁储存均正常或增加。所有10例患者的%HRC持续升高,随后另一例RA患者也出现了%HRC升高,该患者后来呼吸道感染后MDS完全缓解,%HRC恢复正常。在11例接受检测的MDS患者中,发现%HRC与红细胞锌原卟啉水平之间存在强正相关(p=0.01),这表明功能性缺铁导致%HRC升高的MDS病例中红细胞生成无效。7例%HRC升高的患者中有5例在不补充铁的情况下,对人重组促红细胞生成素试验有满意的血红蛋白反应。