Voulgari P V, Chaidos A, Tzouvara E, Alymara V, Alamanos Y, Drosos A A, Bourantas K L
Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, 45110 Ioannina, Greece.
Ann Hematol. 2004 Jan;83(1):22-7. doi: 10.1007/s00277-003-0777-z. Epub 2003 Oct 3.
We evaluated sera from 58 thalassemic patients for the presence of antierythropoietin antibodies to investigate whether these autoantibodies may relate with modest response to treatment with recombinant human erythropoietin (rhEpo). Thirty-seven patients had beta-thalassemia major, 9 patients had beta-thalassemia intermedia, and 12 patients had sickle/beta(+)-thalassemia. Of 58 patients, 24 were on rhEpo treatment in order to increase the intervals between transfusions or the hemoglobin (Hb) values. The study population was divided into three groups according to rhEpo treatment. Group A consisted of 15 patients who were on rhEpo treatment (400-600 IU/kg three times per week, subcutaneously) showing an increase of Hb values or reduction of transfusion requirements. Group B included 9 patients who did not respond to rhEpo and group C consisted of 34 patients who did not receive rhEpo. Laboratory studies included a complete blood count, measurement of serum erythropoietin, immunological evaluation, and determination of antierythropoietin antibodies using enzyme-linked immunosorbent assay (ELISA). There were no significant differences among groups A, B, and C concerning age, Hb, and endogenous erythropoietin values. Fifteen patients had positive antinuclear antibodies and three patients had positive rheumatoid factor. Antierythropoietin antibodies were detected in the sera of seven patients (five men and two women) who received rhEpo. The male patients and one female patient had no response to rhEpo while the other female patient showed response when the dose increased. Other autoantibodies seem to have no clinical significance. In the present study, we detected for the first time in thalassemia patients the presence of antierythropoietin antibodies, which may contribute to rhEpo resistance. Thalassemia patients with low response rates to rhEpo should be evaluated for the presence of antierythropoietin antibodies.
我们评估了58例地中海贫血患者的血清中是否存在抗促红细胞生成素抗体，以研究这些自身抗体是否可能与重组人促红细胞生成素（rhEpo）治疗反应适度有关。37例患者为重型β地中海贫血，9例为中间型β地中海贫血，12例为镰状/β（+）地中海贫血。58例患者中，24例正在接受rhEpo治疗，以延长输血间隔或提高血红蛋白（Hb）值。根据rhEpo治疗情况，将研究人群分为三组。A组由15例接受rhEpo治疗（皮下注射，400 - 600 IU/kg，每周三次）的患者组成，这些患者的Hb值升高或输血需求减少。B组包括9例对rhEpo无反应的患者，C组由34例未接受rhEpo治疗的患者组成。实验室检查包括全血细胞计数、血清促红细胞生成素测定、免疫评估以及使用酶联免疫吸附测定（ELISA）法测定抗促红细胞生成素抗体。A、B、C三组在年龄、Hb和内源性促红细胞生成素值方面无显著差异。15例患者抗核抗体阳性，3例患者类风湿因子阳性。在接受rhEpo治疗的7例患者（5例男性和2例女性）血清中检测到抗促红细胞生成素抗体。男性患者和1例女性患者对rhEpo无反应，而另1例女性患者在剂量增加时出现反应。其他自身抗体似乎无临床意义。在本研究中，我们首次在地中海贫血患者中检测到抗促红细胞生成素抗体的存在，这可能是导致rhEpo抵抗的原因。对rhEpo反应率低的地中海贫血患者应评估是否存在抗促红细胞生成素抗体。
