敌百虫致溶血性贫血一例

患儿女,6岁。因面色苍黄、解酱油色尿3d于2002年1月26日入院。入院前4d,家长用敌百虫5g(10片)溶于水里洒在患儿头发中,并用毛巾包裹灭虱子。第2天患儿出现头昏、乏力,偶诉腹痛,并很快出现面色苍黄,酱油色尿。在当地输“青霉素、解磷定”等,效果不明显。入院前1d,体温39℃,不能站立,意识不清;小便失禁,尿量进行性减少,仍呈酱油色。到当地医院诊治,查乙肝标志物均示阴性,肝、肾功能异常,血常规示重度贫血,急来我院诊治。     

Minocycline-induced hemolytic anemia

A case of drug-induced immune hemolytic anemia is described. A 2 year old boy exhibited sudden anemia and hemoglobinuria after administration of minocycline (MINO). The specific immunoglobulin G antibody against MINO was demonstrated in the patient's serum by western blotting. This is a rare example where anti-minocycline immune complex-mediated hemolysis was responsible for an intravascular hemolytic process.     

Autoimmune hemolytic anemia

Immune hemolytic anemia can be either isoimmune or autoimmune. Autoimmune hemolytic anemias (AIHA) consist of group of disorders whose common characteristics are the presence of an antibody which in turn causes short red blood cell (RBC) life. The rate and site of hemolysis and hence the clinical manifestations depends on the type of antibody attached and its propensity to fix complement. Antibodies of the IgG class are most commonly responsible for AIHA in chidren. Rh erythrocyte antigen is involved in more than 70% of cases. Since the antibody has its maximal activity at 37°C, the resultant hemolysis is called warm antibody induced hemolytic anemia. This is a severe life threatening condition, the clinical features are : sudden onset of pallor, jaundice and dark urine. The cornerstone of diagnosis is a positive Coomb’s antiglobulin test in the presence of hemolysis. Coomb’s test has false negative and false positive rates in about 2–4% and 8% of all cases respectively. The modalities for treatment of warm AIHA include blood transfusion, steroid therapy, intravenous gammaglobulin, plasma-pheresis and splenectomy. The choice depends on the severity of the disease and child’s response to therapy.     

Autoimmune hemolytic anemia

Objective  To study the clinico-hematological profile and treatment outcome in children suffering from auto immune hemolytic anemia (AIHA). Methods  Twelve children were diagnosed with auto immune hemolytic anemia over a period of four years. Direct antiglobulin test was positive in all the cases. Other causes of hemolytic anemia like thalassemia syndromes, hereditary spherocytosis, G6PD deficiency were excluded by appropriate tests. The children were followed up for 6 months to 4 years. Results  The age ranged from 7 mth to 9 yr with a mean age of 4.51 yr. All patients had pallor as the presenting complaint followed by splenomegaly (83.3%), jaundice (66.7%), fever (50%) and bleeding manifestations (16.7%). 9 patients had primary disease and 3 had secondary disease. Tubercular infection was seen in 2 patients with secondary disease. Jaundice was seen equally in both the groups. Oral prednisolone produced remission in 83.3% cases. 4 patients (3 in primary and one in secondary group) had relapse after initial response. All responded to a second course of steroids but had subsequent relapses and developed a chronic course. Conclusion  Autoimmune hemolytic anemia is an uncommon cause of hemolytic anemia in children. Tubercular infection is an underlying pathology in cases of secondary autoimmune hemolytic anemia. Although oral steroids induce remission in most of the cases, relapses are common.     

以自体免疫溶血性贫血为首发表现的系统性红斑狼疮1例

患儿 ,女 ,8岁 ,汉族 ,甘肃省籍 ,因贫血 4个月余入院。患儿无明显诱因出现发热 ,予抗感染治疗后效果欠佳。头晕、头痛症状渐明显 ,出现面色萎黄 ,当地医院查血Hb4 8g/L,尿潜血 ,蛋白 ,酮体 ;直接抗体、抗IgG、抗 C3 、抗自身红细胞抗体阳性 ,诊断为自体免疫溶血性贫血。予人血丙种球蛋白 5 g连用 5d ,泼尼松 5 0mg/d使用 6周后逐渐减量。用药后患儿Hb升至 170 g/L。激素减量后患儿Hb进行性下降 ,为进一步诊治来我院。查体 :中度贫血貌 ,无蝶型红斑 ,浅表淋巴结不大 ,心肺正常 ,肝脾不大。血WBC8.6× 10 9/L ,PLT 2 6 0× 10 9/…     

Pulmonary veno-occlusive disease associated with microangiopathic hemolytic anemia and chemotherapy of gastric adenocarcinoma

A 68-year-old man was treated with FAM (5-fluorouracil, doxorubicin [Adriamycin], mitomycin-C) for metastic gastric adenocarcinoma. Twelve months later while in complete clinical remission, pulmonary hypertension and microangiopathic hemolytic anemia were recognized, progressed, and ended in his demise 6 months later. At necropsy, minimal residual cancer and severe pulmonary veno-occlusive disease was found. Pulmonary veno-occlusive disease may occur in association with microangiopathic hemolytic anemia and cancer chemotherapy.     

Autoimmune hemolytic anemia in children

The clinical and hematological profile and treatment outcome of children with warm autoimmune hemolytic anemia (AIHA) were assessed using retrospective case record analysis. There were 26 (17 idiopathic; 9 secondary) patients with a median age of 11 years. Pallor (100%), fever (39%), and jaundice (59%) were the main presenting complaints. Jaundice was much more common in idiopathic (70%) compared to secondary (44%). Direct antiglobulin test was negative in 3 patients. Oral prednisolone produced remission in 81% patients. Four patients relapsed after a median period of 7 months (2 months to 2 year) after response. All responded to a second course of steroids in median 14 days. One child required cyclosporin A in addition. No correlation was found between response and parameters such as age, sex, jaundice, low pretreatment hemoglobin, reticulocyte count, total leukocyte count, platelet count, subtype of AIHA, and hepatosplenomegaly. Relapse correlated with increased duration between the onset of symptoms and treatment. This study indicates that oral prednisolone is an effective therapy for autoimmune hemolytic anemia. In refractory cases cyclosporine A may be useful.