Ah类孟买表型的血型差异:一种罕见血型变异及其临床意义。

Q4 Medicine
M S Bhagavathi, N Das, S Prakash, A Sahu, S Routray, S Mukherjee
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引用次数: 1

摘要

具有罕见的类孟买表型的个体具有与FUT1和/或FUT2基因相关的产生H的遗传缺陷。我们报告一例血型差异的准孟买病人从三级护理医院的印度东部。31岁女性风湿性心脏病患者表现为疲劳和呼吸困难,随后安排进行瓣膜成形术,为此向血液中心提出输血请求。在输血前检测中,红细胞(RBC)检测显示为O型,血清检测显示与B型红细胞反应性强,与O型红细胞反应性弱,与A型红细胞反应性极弱。唾液抑制试验和红细胞酶治疗表明患者为“类孟买Ah”表型。患者Lewis表型为Le(a-b+)。该患者血清中除抗b外,还存在冷反应性抗ih。本病例报告强调了当常规ABO分型(使用正向和反向分组)不确定时,进行高级免疫血液学检查的重要性,包括吸附、洗脱、酶治疗和唾液抑制试验,以确定弱A或B亚群以及罕见的类孟买血型。准确识别血型有助于预防与输血有关的不良事件和鼓励安全输血做法。具有罕见的类孟买表型的个体具有与FUT1和/或FUT2基因相关的产生H的遗传缺陷。我们报告一例血型差异的准孟买病人从三级护理医院的印度东部。31岁女性风湿性心脏病患者表现为疲劳和呼吸困难,随后安排进行瓣膜成形术,为此向血液中心提出输血请求。在输血前检测中,红细胞(RBC)检测显示为O型,血清检测显示与B型红细胞反应性强,与O型红细胞反应性弱,与A型红细胞反应性极弱。唾液抑制试验和红细胞酶治疗表明患者为“类孟买Ah”表型。患者Lewis表型为Le(a-b +)。该患者血清中除抗b外,还存在冷反应性抗ih。本病例报告强调了当常规ABO分型(使用正向和反向分组)不确定时,进行高级免疫血液学检查的重要性,包括吸附、洗脱、酶治疗和唾液抑制试验,以确定弱A或B亚群以及罕见的类孟买血型。准确识别血型有助于预防与输血有关的不良事件和鼓励安全输血做法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Blood group discrepancy in Ah para-Bombay phenotype: a rare blood group variant and its clinical significance.

Individuals with the rare para-Bombay phenotype have inherited defects in producing H associated with FUT1 and/or FUT2 genes. We report a case of blood group discrepancy in a para-Bombay patient from a tertiary care hospital of eastern India. A 31-year-old woman with rheumatic heart disease presented with fatigue and breathlessness and was then scheduled for valvuloplasty, for which a blood transfusion request was sent to the blood center. During pre-transfusion testing, red blood cell (RBC) testing showed group O, and serum testing showed strong reactivity with group B RBCs, weak reactivity with group O RBCs, and very weak reactivity with group A RBCs. Saliva inhibition testing and enzyme treatment of RBCs concluded the patient to be of "Ah para-Bombay" phenotype. The patient's Lewis phenotype was Le(a-b+). This patient's serum also had cold-reacting anti-IH along with anti-B. This case report highlights the importance of performing an advanced immunohematologic workup, including adsorption, elution, enzyme treatment, and saliva inhibition testing for identification of weak A or B subgroups as well as the rare para-Bombay blood group, when routine ABO typing, using forward and reverse grouping, is inconclusive. Accurate identification of blood group helps in preventing transfusion-related adverse events and encouraging safe transfusion practice.

Individuals with the rare para-Bombay phenotype have inherited defects in producing H associated with FUT1 and/or FUT2 genes. We report a case of blood group discrepancy in a para-Bombay patient from a tertiary care hospital of eastern India. A 31-year-old woman with rheumatic heart disease presented with fatigue and breathlessness and was then scheduled for valvuloplasty, for which a blood transfusion request was sent to the blood center. During pre-transfusion testing, red blood cell (RBC) testing showed group O, and serum testing showed strong reactivity with group B RBCs, weak reactivity with group O RBCs, and very weak reactivity with group A RBCs. Saliva inhibition testing and enzyme treatment of RBCs concluded the patient to be of “Ah para-Bombay” phenotype. The patient’s Lewis phenotype was Le(a–b+). This patient’s serum also had cold-reacting anti-IH along with anti-B. This case report highlights the importance of performing an advanced immunohematologic workup, including adsorption, elution, enzyme treatment, and saliva inhibition testing for identification of weak A or B subgroups as well as the rare para-Bombay blood group, when routine ABO typing, using forward and reverse grouping, is inconclusive. Accurate identification of blood group helps in preventing transfusion-related adverse events and encouraging safe transfusion practice.

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来源期刊
Immunohematology
Immunohematology Medicine-Medicine (all)
CiteScore
1.30
自引率
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发文量
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