{"title":"注意温度变化:阵发性寒性血红蛋白尿1例报告","authors":"","doi":"10.24966/hbtd-2999/100021","DOIUrl":null,"url":null,"abstract":"Paroxysmal cold haemoglobinuria is an immune haemolytic syndrome characterized by the presence of autoantibodies reactive against specific red blood cell antigens. At low temperatures, these antibodies and the complement fix to the red blood cells, leading to haemolysis upon warming up. We present the case of a twenty-one months old male child, who presented with a six-day history of malaise, productive cough and fe -ver, already medicated with amoxicillin and clavulanic acid two days before. On physical examination, the child was prostrated, severe ly pale, but with no hemodynamic instability or difficulty breathing. Blood tests revealed the presence of severe anaemia (Hb 5.4 g/dL), leucocytosis (19.90 x 10 9 /L), without immature cells in the blood film; reticulocytosis (89.7 x 10 6 /L) and evidence of hemolysis (haptoglo-bin <8 mg/dL; LDH 1328 UI/L; bilirubin 1 mg/dL and unconjugated bilirubin 0.6 mg/dL), C-reactive-protein 56.7 mg/L. He received a transfusion of red blood cells, initiated treatment with oral clarithro mycin and reinforced the rewarming of the extremities with good re - sults (Hb 9.6 g/dL). Regarding serological investigation, the results revealed a positive IgM for Mycoplasma pneumoniae ; positive Direct Antiglobulin Test with specificity for CD3, and positive Donath Land steiner test, leading to the diagnosis. This case report represents a syndrome that even though rare, is important to bear in mind when facing a case of severe haemolysis. Despite the favorable outcome, additional follow-up must be placed in outpatient setting, with periodic clinical and laboratorial revalua -tions.","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"31 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2019-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":"{\"title\":\"Beware Of Temperature Changes: A Case Report Of Paroxysmal Cold Haemoglobinuria\",\"authors\":\"\",\"doi\":\"10.24966/hbtd-2999/100021\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Paroxysmal cold haemoglobinuria is an immune haemolytic syndrome characterized by the presence of autoantibodies reactive against specific red blood cell antigens. At low temperatures, these antibodies and the complement fix to the red blood cells, leading to haemolysis upon warming up. We present the case of a twenty-one months old male child, who presented with a six-day history of malaise, productive cough and fe -ver, already medicated with amoxicillin and clavulanic acid two days before. On physical examination, the child was prostrated, severe ly pale, but with no hemodynamic instability or difficulty breathing. Blood tests revealed the presence of severe anaemia (Hb 5.4 g/dL), leucocytosis (19.90 x 10 9 /L), without immature cells in the blood film; reticulocytosis (89.7 x 10 6 /L) and evidence of hemolysis (haptoglo-bin <8 mg/dL; LDH 1328 UI/L; bilirubin 1 mg/dL and unconjugated bilirubin 0.6 mg/dL), C-reactive-protein 56.7 mg/L. He received a transfusion of red blood cells, initiated treatment with oral clarithro mycin and reinforced the rewarming of the extremities with good re - sults (Hb 9.6 g/dL). Regarding serological investigation, the results revealed a positive IgM for Mycoplasma pneumoniae ; positive Direct Antiglobulin Test with specificity for CD3, and positive Donath Land steiner test, leading to the diagnosis. This case report represents a syndrome that even though rare, is important to bear in mind when facing a case of severe haemolysis. Despite the favorable outcome, additional follow-up must be placed in outpatient setting, with periodic clinical and laboratorial revalua -tions.\",\"PeriodicalId\":236918,\"journal\":{\"name\":\"Hematology, Blood Transfusion and Disorders\",\"volume\":\"31 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-11-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hematology, Blood Transfusion and Disorders\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.24966/hbtd-2999/100021\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hematology, Blood Transfusion and Disorders","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.24966/hbtd-2999/100021","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 1
摘要
阵发性寒性血红蛋白尿是一种免疫性溶血综合征,其特征是存在针对特定红细胞抗原的自身抗体。在低温下,这些抗体和补体固定在红细胞上,在升温时导致溶血。我们报告一个21个月大的男婴,他有6天的不适史,咳嗽和发烧,两天前已经服用了阿莫西林和克拉维酸。体格检查时,患儿俯卧,面色严重苍白,但无血流动力学不稳定或呼吸困难。血液检查显示存在严重贫血(血红蛋白5.4 g/dL),白细胞增多(19.90 x 10 9 /L),血膜中未见未成熟细胞;网织红细胞增多症(89.7 × 10.6 /L)和溶血症状(haptoglob -bin < 8mg /dL;LDH 1328ui / l;胆红素1 mg/dL和未结合胆红素0.6 mg/dL), c -反应蛋白56.7 mg/L。他接受了红细胞输注,开始口服克拉霉素治疗,并加强了四肢的复温,结果良好(Hb 9.6 g/dL)。血清学检查结果显示肺炎支原体IgM阳性;CD3特异性直接抗球蛋白试验阳性,Donath Land steiner试验阳性,导致诊断。本病例报告代表了一种综合征,即使罕见,重要的是要记住,当面对严重溶血的情况下。尽管结果良好,但必须在门诊进行额外的随访,定期进行临床和实验室评估。
Beware Of Temperature Changes: A Case Report Of Paroxysmal Cold Haemoglobinuria
Paroxysmal cold haemoglobinuria is an immune haemolytic syndrome characterized by the presence of autoantibodies reactive against specific red blood cell antigens. At low temperatures, these antibodies and the complement fix to the red blood cells, leading to haemolysis upon warming up. We present the case of a twenty-one months old male child, who presented with a six-day history of malaise, productive cough and fe -ver, already medicated with amoxicillin and clavulanic acid two days before. On physical examination, the child was prostrated, severe ly pale, but with no hemodynamic instability or difficulty breathing. Blood tests revealed the presence of severe anaemia (Hb 5.4 g/dL), leucocytosis (19.90 x 10 9 /L), without immature cells in the blood film; reticulocytosis (89.7 x 10 6 /L) and evidence of hemolysis (haptoglo-bin <8 mg/dL; LDH 1328 UI/L; bilirubin 1 mg/dL and unconjugated bilirubin 0.6 mg/dL), C-reactive-protein 56.7 mg/L. He received a transfusion of red blood cells, initiated treatment with oral clarithro mycin and reinforced the rewarming of the extremities with good re - sults (Hb 9.6 g/dL). Regarding serological investigation, the results revealed a positive IgM for Mycoplasma pneumoniae ; positive Direct Antiglobulin Test with specificity for CD3, and positive Donath Land steiner test, leading to the diagnosis. This case report represents a syndrome that even though rare, is important to bear in mind when facing a case of severe haemolysis. Despite the favorable outcome, additional follow-up must be placed in outpatient setting, with periodic clinical and laboratorial revalua -tions.