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一名重型β地中海贫血患者发生广泛的全身血栓栓塞,包括酷似心房黏液瘤的心脏内血栓形成:病例报告。

Extensive systemic thrombo-embolism including intra-cardiac thrombosis mimicking an atrial myxoma in a patient with beta thalassaemia major - a case report.

机构信息

University Medical Unit, North Colombo (Teaching) Hospital, Ragama, Sri Lanka.

Cardiology Unit, North Colombo (Teaching) Hospital, Ragama, Sri Lanka.

出版信息

BMC Cardiovasc Disord. 2023 Oct 31;23(1):532. doi: 10.1186/s12872-023-03576-2.

DOI:10.1186/s12872-023-03576-2
PMID:37907862
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10619236/
Abstract

BACKGROUND

Sepsis and thrombo-embolic disease are well known complications of thalassemia major. Intracardiac thrombi are however rare and can lead to diagnostic dilemmas.

CASE PRESENTATION

We report the case of a 20-year-old female splenectomised thalassaemia major patient with severe iron overload, who presented with life threatening sepsis associated with a liver abscess. Discovery of a large oscillating intra cardiac lesion on 2D echocardiogram confirmed by Contrast Enhanced Computed Tomography (CECT) chest in the right atrium extending from the left hepatic vein through the inferior vena cava complicated the clinical course. After a prolonged Intensive Care Unit (ICU) stay supported with antibiotics and anticoagulation, she recovered with evidence of resolution of the intra cardiac thrombus.

CONCLUSIONS

Early recognition and prompt aggressive treatment of sepsis in patients with thalassemia is essential to prevent complications. Intracardiac thrombosis is a potentially treatable cause for an intra cardiac mass in patients with thalassemia major, which should not be missed.

摘要

背景

败血症和血栓栓塞性疾病是重型地中海贫血的已知并发症。然而,心脏内血栓很罕见,但可导致诊断难题。

病例介绍

我们报告了 1 例 20 岁女性,曾行脾切除术的重型地中海贫血患者,存在严重铁过载,因危及生命的败血症合并肝脓肿就诊。二维超声心动图发现一个大的摆动性心内病变,经胸部对比增强计算机断层扫描(CECT)证实,在右心房从左肝静脉延伸至下腔静脉,使临床病程复杂化。在重症监护病房(ICU)经过长时间支持治疗,包括抗生素和抗凝治疗后,她恢复了,心内血栓得到了缓解。

结论

早期识别和积极治疗重型地中海贫血患者的败血症至关重要,可预防并发症。心内血栓是重型地中海贫血患者心内肿块的一种潜在可治疗病因,不应漏诊。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a35b/10619236/4d0ee8a436ee/12872_2023_3576_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a35b/10619236/4483727f693a/12872_2023_3576_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a35b/10619236/4d0ee8a436ee/12872_2023_3576_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a35b/10619236/4483727f693a/12872_2023_3576_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a35b/10619236/4d0ee8a436ee/12872_2023_3576_Fig2_HTML.jpg

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J Clin Med. 2021 Dec 21;11(1):15. doi: 10.3390/jcm11010015.
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The changing epidemiology of the ageing thalassaemia populations: A position statement of the Thalassaemia International Federation.衰老地中海贫血人群的流行病学变化:地中海贫血国际联合会立场声明。
Eur J Haematol. 2020 Jul;105(1):16-23. doi: 10.1111/ejh.13410. Epub 2020 Apr 15.
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Challenges of blood transfusions in β-thalassemia.
β-地中海贫血症输血面临的挑战。
Blood Rev. 2019 Sep;37:100588. doi: 10.1016/j.blre.2019.100588. Epub 2019 Jul 6.
4
Klebsiella pneumoniae Multiple Liver Abscesses and Bacteremia in a Transfusion-dependent β Thalassemia Major Patient.一名依赖输血的重型β地中海贫血患者发生肺炎克雷伯菌多发性肝脓肿和菌血症
J Pediatr Hematol Oncol. 2020 Aug;42(6):e497-e498. doi: 10.1097/MPH.0000000000001541.
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Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population.在一个依赖输血的希腊β地中海贫血患者的大队列中进行生存分析,并与普通人群进行死亡率比较。
Eur J Haematol. 2011 Apr;86(4):332-8. doi: 10.1111/j.1600-0609.2011.01582.x.
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Beta-thalassemia.β-地中海贫血。
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