Sharma R A, Hiwarkar P, Manglani M V, Muralidhar H P
Division of Paediatric Haematology-Oncology, L.T.M.G. Hospital, Sion, Mumbai - 400022, India.
J Postgrad Med. 2002 Jan-Mar;48(1):37-8.
Primary acquired pure red cell aplasia is a rare occurrence in childhood. An eleven-year old boy presented to us with pallor, which required multiple packed red cell transfusions. He did not have hepatosplenomegaly, jaundice or lymphadenopathy. Bone marrow examination revealed the diagnosis of pure red cell aplasia. All possible investigations were done to exclude secondary causes of pure red cell aplasia. No secondary cause was found on investigations. Rheumatoid factor and anti-nuclear antibodies were positive. He was started on oral steroids, to which he did not respond. He was then given cyclosporine A. Response to cyclosporine was dramatic and the child now does not require any transfusions.
原发性获得性纯红细胞再生障碍在儿童时期较为罕见。一名11岁男孩因面色苍白前来就诊,需要多次输注浓缩红细胞。他没有肝脾肿大、黄疸或淋巴结病。骨髓检查确诊为纯红细胞再生障碍。进行了所有可能的检查以排除纯红细胞再生障碍的继发性病因。检查未发现继发性病因。类风湿因子和抗核抗体呈阳性。他开始口服类固醇治疗,但没有反应。随后给他使用环孢素A。对环孢素的反应显著,该患儿现在不需要任何输血治疗。