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一名中间型β地中海贫血患者发生胸腔内髓外造血相关胸腔积液病例及文献综述

A case of intrathoracic extramedullary hematopoiesis-associated pleural effusion in a beta-thalassemia intermedia patient and a short literature review.

作者信息

Barakos Georgios Petros, Papazafiropoulou Athanasia, Mihos Konstantinos, Eminidou Valeria, Andronikou Stefani, Ainalidou Lemonia, Mofori Niki, Gkraikou Themis, Athanasakis Georgios, Papanas Nikolaos, Antonopoulos Stavros

机构信息

First Department of Internal Medicine, "Tzaneio" General Hospital of Piraeus, Piraeus, Greece.

Second Department of Internal Medicine, Democritus University of Thrace, Alexandroupolis, Greece.

出版信息

SAGE Open Med Case Rep. 2024 Dec 4;12:2050313X241305169. doi: 10.1177/2050313X241305169. eCollection 2024.

DOI:10.1177/2050313X241305169
PMID:39634073
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11615976/
Abstract

Extramedullary hematopoiesis is a phenomenon that occurs in conditions of ineffective bone marrow function. In the context of thalassemias, extramedullary hematopoiesis is more frequently observed in beta-thalassemia intermedia patients, with thoracic paravertebral extramedullary hematopoiesis being relatively common. However, extramedullary hematopoiesis-related pleural effusion is a rare occurrence. Herein, we report a 43-year-old female patient, who presented with worsening dyspnea and a massive pleural effusion. Her medical history includes beta-thalassemia intermedia with known paravertebral extramedullary hematopoiesis sites and a laparoscopic cholecystectomy a month ago. The absence of pleural fluid from recent prior imaging pointed toward the rapid accumulation of fluid. After a thorough diagnostic workup, we attributed the effusion to thoracic paravertebral extramedullary hematopoiesis. The patient was effectively treated with drainage of the effusion after thoracentesis and antibiotics. On follow-up, she had a stable radiographic image without pleural fluid recurrence. Extramedullary hematopoiesis-associated pleural effusion should be in the differential diagnosis of pleural effusion, in cases of compatible medical history.

摘要

髓外造血是一种在骨髓功能无效的情况下发生的现象。在地中海贫血的背景下,髓外造血在中间型β地中海贫血患者中更常见,胸段椎旁髓外造血相对常见。然而,与髓外造血相关的胸腔积液是一种罕见的情况。在此,我们报告一名43岁女性患者,她出现进行性加重的呼吸困难和大量胸腔积液。她的病史包括中间型β地中海贫血,已知有椎旁髓外造血部位,以及一个月前的腹腔镜胆囊切除术。近期先前影像学检查未发现胸腔积液提示液体迅速积聚。经过全面的诊断检查,我们将胸腔积液归因于胸段椎旁髓外造血。患者在胸腔穿刺引流积液并使用抗生素后得到有效治疗。随访时,她的影像学图像稳定,无胸腔积液复发。在有相关病史的情况下,髓外造血相关胸腔积液应列入胸腔积液的鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f256/11615976/6eb990aaeaf5/10.1177_2050313X241305169-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f256/11615976/44efa8abdff0/10.1177_2050313X241305169-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f256/11615976/6eb990aaeaf5/10.1177_2050313X241305169-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f256/11615976/44efa8abdff0/10.1177_2050313X241305169-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f256/11615976/6eb990aaeaf5/10.1177_2050313X241305169-fig2.jpg

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Extramedullary hematopoiesis in cancer.癌症中的髓外造血。
Exp Mol Med. 2024 Mar;56(3):549-558. doi: 10.1038/s12276-024-01192-4. Epub 2024 Mar 5.
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Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report.肋骨骨髓外造血及中间型β-地中海贫血后严重肺动脉高压:病例报告。
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Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review.
输血依赖型β地中海贫血(TDT)患者的骨髓外造血:系统评价。
Ann Med. 2022 Dec;54(1):764-774. doi: 10.1080/07853890.2022.2048065.
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A 43-Year-Old Man With Rapidly Respiratory Failure and Spontaneous Massive Hemothorax.一名43岁男性,伴有快速呼吸衰竭和自发性大量血胸。
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