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过去认为不稳定血红蛋白病(uHb)临床少见,近年来由于对该病的认识及诊断技术水平的提高,发现本病并不少见,其发病年龄及临床表现变化很大,且常在感染或服用氧化性质药物后出现溶血或使原有的贫血加重,应予以重视。我院近些年来诊治4例,现报道于下。 例1、女,40岁,平时无何不适,因劳累后突发苍白、高热,全身皮肤出血点及淤斑2月入院;10年前曾于感冒后出现贫血,经输血等治疗贫血纠正。体格检查:面色苍白,全身皮肤散在出血点及淤斑、表浅淋巴结不大,心肺无特殊,肝脾未扪及。实验室检查:Hb50g/L,WBC0.9×10~9/L,成熟红细胞呈大红细胞改变,外周血可见有核红细胞,网织红细
In the past, unstable hemoglobin disease (uHb) was clinically uncommon. In recent years, due to the increased understanding of the disease and the improvement of diagnostic techniques, it is not uncommon to find that the disease is very variable in age and clinical manifestations, and is often infected or taken Oxidation of the drug after hemolysis or make the original anemia, should be taken seriously. In recent years, our hospital diagnosis and treatment of 4 cases, are reported below. Case 1, female, 40 years old, usually no discomfort, due to fatigue after the sudden pale, high fever, systemic skin bleeding and ecchymosis admitted in February; 10 years ago in a cold anemia, transfusion and other anemia correction. Physical examination: pale, whole body scattered in the bleeding spots and ecchymosis, superficial lymph nodes is small, no special cardiopulmonary, liver and spleen not palpable. Laboratory tests: Hb50g / L, WBC0.9 × 10 ~ 9 / L, mature red blood cells showed changes in red blood cells, peripheral blood visible nucleated red blood cells, reticulocyte red