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作者首次报道了一罕见骨髓增生异常综合征(MDS)伴获得性Ⅷ:C 抑制物的病例。患者,女,75岁。2个月前发现贫血,当时Hb 为6.1g/dl 且曾输血。最近自发性皮下出血入院。69岁时由于股骨颈骨折手术治疗,无出血并发症。体检:躯干及四肢广泛皮下出血、左臀部有一直径15cm 的血肿。苍白明显,无黄疸。化验:Hb 6.9g/dl,末梢血涂片RBC 形态一部分正常,一部分为大红细胞,未见中性粒细胞分叶过多现象。骨髓增生活跃,以红系为主。可见巨幼红细胞和红系增生异常。Perl’s 染色显示许多病理性环状铁粒幼细胞,占幼稚红细胞中40%。粒细胞和血小板生成正常。诊断:MDS-RAS。活化的部分凝血活酶时间(APTT)71秒(对照37秒),用正常混合血浆不能纠正,提示存在抑制物。血浆凝血酶原时间和纤维
The authors first reported a rare case of myelodysplastic syndrome (MDS) with acquired Ⅷ: C inhibitors. Patient, female, 75 years old. Anemia was found 2 months ago when Hb was 6.1 g / dl and had blood transfusions. Recent spontaneous subcutaneous bleeding admitted to hospital. 69 years old due to femoral neck fracture surgery, no bleeding complications. Physical examination: extensive subcutaneous hemorrhage in the trunk and limbs, hematoma with a diameter of 15 cm in the left buttocks. Pale, no jaundice. Assay: Hb 6.9g / dl, peripheral blood smear RBC morphology part of the normal, part of the red blood cells, no neutrophil lobulation phenomenon. Bone marrow hyperplasia, mainly erythroid. Megaloblastic red cells and erythroid hyperplasia can be seen. Perl’s staining showed many pathological ring-shaped mitochondria, accounting for 40% of immature red blood cells. Granulocytes and platelets produce normal. Diagnosis: MDS-RAS. Activation of the partial thromboplastin time (APTT) 71 seconds (control 37 seconds), with normal mixed plasma can not be corrected, suggesting the presence of inhibitors. Plasma prothrombin time and fiber