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继发于类风湿关节炎的纯红细胞再生障碍:一例报告。

Pure red cell aplasia secondary to rheumatoid arthritis: a case report.

机构信息

Teaching Hospital, Peradeniya, Sri Lanka.

Department of Medicine, Faculty of Medicine, University of Peradeniya, Kandy, Sri Lanka.

出版信息

J Med Case Rep. 2021 Dec 6;15(1):578. doi: 10.1186/s13256-021-03141-5.

Abstract

BACKGROUND

Rheumatoid arthritis is a common autoimmune disease with many extra-articular manifestations. Pure red cell aplasia is a rare manifestation of rheumatoid arthritis and is sparsely documented in the literature, with a variable clinical outcome following immunosuppressive therapy.

CASE PRESENTATION

A 63-year-old Sinhalese female presented with transfusion-dependent anemia associated with deforming inflammatory arthritis. She also had leukopenia, right subclavian venous thrombosis, and generalized lymphadenopathy. The diagnosis of rheumatoid arthritis following initial clinical workup and additional blood and bone marrow investigations revealed pure red cell aplasia as a secondary manifestation of rheumatoid arthritis after excluding other secondary causes, such as infections, thymoma, thrombophilic conditions, and hematological malignancy. She responded well to oral prednisolone, cyclosporine A, and hydroxychloroquine, and she attained complete recovery in 2 months.

CONCLUSION

Pure red cell aplasia is a disabling illness that may lead to transfusion-dependent anemia, which may occur due to rare extrapulmonary manifestation of rheumatoid arthritis. The diagnosis of pure red cell aplasia secondary to rheumatoid arthritis may be challenging where hematological investigations, including bone marrow biopsy, will aid in the diagnosis, and early diagnosis and treatment will bring about a better outcome.

摘要

背景

类风湿关节炎是一种常见的自身免疫性疾病,有许多关节外表现。纯红细胞再生障碍性贫血是类风湿关节炎的一种罕见表现,文献记载较少,免疫抑制治疗后的临床结局不一。

病例介绍

一位 63 岁的僧伽罗女性,表现为依赖输血的贫血症和变形性炎性关节炎。她还伴有白细胞减少症、右锁骨下静脉血栓形成和全身淋巴结病。经过初始临床检查和其他血液及骨髓检查,诊断为类风湿关节炎,同时排除了其他继发性病因,如感染、胸腺瘤、血栓形成倾向和血液恶性肿瘤等,随后发现纯红细胞再生障碍性贫血是类风湿关节炎的继发表现。她对口服泼尼松龙、环孢素 A 和羟氯喹反应良好,2 个月内完全康复。

结论

纯红细胞再生障碍性贫血是一种致残性疾病,可导致依赖输血的贫血症,可能由类风湿关节炎罕见的肺外表现引起。类风湿关节炎继发的纯红细胞再生障碍性贫血的诊断可能具有挑战性,需要进行血液学检查,包括骨髓活检,以协助诊断,早期诊断和治疗将带来更好的结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0da6/8647461/44f1ce924725/13256_2021_3141_Fig1_HTML.jpg

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