混合性结缔组织病伴继发性免疫性血小板减少性紫癜1例

S. Rupasinghe, W. Kularatne
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摘要

混合性结缔组织病(MCTD)是一种由抗u1核糖核蛋白(RNP)抗体证实的全身性结缔组织疾病。MCTD的病因尚不清楚。我们提出一个病例的年轻女性表现为月经过多,症状性贫血,小关节疼痛和雷诺现象。她被发现有血小板减少症,抗u1rnp抗体阳性。她被诊断为MCTD合并肺动脉高压和继发性免疫性血小板减少性紫癜。她接受了皮质类固醇和免疫抑制剂治疗,病情完全缓解。尽管继发性ITP与自身免疫性疾病有关,但文献中MCTD合并继发性ITP的病例很少。以出血为表现的MCTD病例也很少见。这应该被认为是一个鉴别诊断,在病人表现出出血的表现,并提示潜在的结缔组织疾病的症状。早期诊断和治疗可改善预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Mixed connective tissue disease with secondary immune thrombocytopenic purpura -- a case report
Mixed connective tissue disease [MCTD] is a generalized connective tissue disease which is confirmed by the presence of anti-U1 ribonucleoprotein (RNP) antibodies. The etiology of MCTD remains unclear. We present a case of a young female presenting with menorrhagia, symptomatic anaemia, small joint pain and raynaud’s phenomenon. She was found to have thrombocytopenia with positive anti-U1RNP antibodies. She was diagnosed as having MCTD with pulmonary hypertension and secondary immune thrombocytopenic purpura [ITP]. She was treated with corticosteroids and immunosuppressants which led to complete remission. Even though secondary ITP is associated with autoimmune diseases, cases of MCTD with secondary ITP are rare in literature. Cases of MCTD presenting with bleeding manifestations are rare as well. This should be considered as a differential diagnosis in a patient presenting with bleeding manifestations along with symptoms suggestive of an underlying connective tissue disorder. Early diagnosis and treatment enhance prognosis.
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