输血后反常的血红蛋白下降:伴有巨噬细胞激活综合征的地中海贫血的高溶血。

IF 6.4 4区 医学 Q1 MEDICINE, GENERAL & INTERNAL
Sandeep Rao Kordcal, Sarthak Kain, Amandeep Singh, Manish Soneja, Naval K Vikram, Hem Pandey, Tulika Seth
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引用次数: 0

摘要

高溶血综合征(HHS)是一种罕见的严重输血并发症,其特征是输血后血红蛋白(Hb)水平反常下降,通常低于输血前的值。大多数报告的病例是镰状细胞病患者,在其他血红蛋白病中很少报道。我们报告一例新诊断的δ - β地中海贫血患者的HHS,表现为巨噬细胞激活综合征(MAS),通过静脉注射免疫球蛋白(IVIG)、皮质类固醇、利妥昔单抗和促红细胞生成素成功治疗。19岁女性,输血后3周出现发热、黄疸、肝脾肿大。调查显示小细胞贫血,LDH和间接胆红素升高,高效液相色谱结果提示地中海贫血。她在随后的输血后出现溶血和反常的血红蛋白下降,并伴有网状红细胞减少症。异体免疫最初不存在,但随后检测到抗e和抗k抗体。尽管进行了抗原匹配的输血,但溶血现象仍然存在,这引起了对HHS的怀疑。患者接受IVIG、甲基强的松龙、促红细胞生成素和利妥昔单抗治疗,导致血红蛋白水平最初改善,随后再次下降。随后,患者出现发热和白细胞增多,但无感染迹象,高HScore提示怀疑为巨噬细胞激活综合征(MAS)。开始使用地塞米松,导致退热,网织红细胞计数正常化,血红蛋白显著升高。出院时病情稳定,随访6个月。本病例强调高溶血综合征(HHS)是血红蛋白病患者的潜在并发症。虽然其确切的发病机制尚不清楚,但巨噬细胞活化似乎起着重要作用。早期识别和及时开始适当的免疫治疗可导致良好的结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Paradoxical Hemoglobin Drop Post-Transfusion: Hyperhemolysis in Delta-Beta Thalassemia with Macrophage Activation Syndrome.

Hyperhaemolysis syndrome (HHS) is a rare, severe complication of transfusion characterised by a paradoxical drop in haemoglobin (Hb) levels post-transfusion, often below pre-transfusion values. Most reported cases are in patients with sickle cell disease and it has been rarely reported in other haemoglobinopathies. We report a case of HHS in a newly diagnosed delta-beta thalassaemia patient, manifesting as macrophage activation syndrome (MAS), successfully managed with intravenous immunoglobulin (IVIG), corticosteroids, rituximab, and erythropoietin. A 19-year-old female presented with fever, jaundice, and hepatosplenomegaly three weeks after a blood transfusion. Investigations revealed microcytic anaemia, elevated LDH and indirect bilirubin, and HPLC findings suggestive of delta-beta thalassaemia. She developed haemolysis and a paradoxical Hb drop after subsequent transfusions, along with reticulocytopenia. Alloimmunisation was initially absent but anti-E and anti-K antibodies were subsequently detected. Despite antigen-matched transfusions, haemolysis persisted, raising suspicion for HHS. She was managed with IVIG, methylprednisolone, erythropoietin, and rituximab, resulting in initial improvement in haemoglobin levels, which subsequently declined again. She then developed fever and leukocytosis without evidence of infection, and a high HScore raised suspicion for macrophage activation syndrome (MAS). Dexamethasone was initiated, leading to defervescence, normalisation of the reticulocyte count, and a significant rise in haemoglobin. She was discharged in stable condition and remained well at 6-month follow-up. This case highlights hyperhaemolysis syndrome (HHS) as a potential complication in patients with haemoglobinopathies. Although its exact pathogenesis remains unclear, macrophage activation appears to play an important role. Early recognition and timely initiation of appropriate immunotherapy can lead to favourable outcomes.

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来源期刊
CiteScore
6.90
自引率
5.30%
发文量
263
审稿时长
4-8 weeks
期刊介绍: QJM, a renowned and reputable general medical journal, has been a prominent source of knowledge in the field of internal medicine. With a steadfast commitment to advancing medical science and practice, it features a selection of rigorously reviewed articles. Released on a monthly basis, QJM encompasses a wide range of article types. These include original papers that contribute innovative research, editorials that offer expert opinions, and reviews that provide comprehensive analyses of specific topics. The journal also presents commentary papers aimed at initiating discussions on controversial subjects and allocates a dedicated section for reader correspondence. In summary, QJM's reputable standing stems from its enduring presence in the medical community, consistent publication schedule, and diverse range of content designed to inform and engage readers.
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