Rau Aarathi R, Usha M, Mallya Pooja, Rau A T K
Department of Pathology, MS Ramaiah Medical College, Bangalore, 560054 India.
Department of Paediatrics, MS Ramaiah Medical College, Bangalore, 560054 India.
Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):433-6. doi: 10.1007/s12288-014-0456-3. Epub 2014 Sep 7.
We describe a sixteen year old with Wilson's disease on copper chelation and subsequent high dose oral zinc who developed severe anemia and neutropenia. Bone marrow aspirate done to evaluate the cause of bicytopenia revealed trilineage dysplasia. Correlating the clinical context with bone marrow and biochemical parameters, copper deficiency was suspected and he was given a trial of therapy, following which the hematological parameters improved. This case highlights hypocupremia as a reversible cause of bone marrow dysplasia in patients with Wilson's disease on chelation, where serum copper levels are not useful in the diagnosis. We also believe that monitoring of the blood counts in patients on copper chelation may provide a clue to impending copper deficiency.
我们描述了一名16岁患有威尔逊病的患者,该患者正在接受铜螯合治疗,随后服用高剂量口服锌,出现了严重贫血和中性粒细胞减少。为评估全血细胞减少的原因而进行的骨髓穿刺显示三系发育异常。将临床情况与骨髓及生化参数相关联后,怀疑是铜缺乏,于是对其进行了试验性治疗,之后血液学参数得到改善。该病例突出了低铜血症是威尔逊病患者接受螯合治疗时骨髓发育异常的一个可逆原因,在此情况下血清铜水平对诊断并无帮助。我们还认为,对接受铜螯合治疗的患者进行血细胞计数监测可能为即将出现的铜缺乏提供线索。