髓外造血表现为胸椎脊髓压迫的年轻成人地中海贫血:1例报告。

IF 0.2 Q3 Medicine
Prasheelkumar Premnarayan Gupta, Salman T Shaikh, Richa Premnarayan Goyal, Deepak Premnarayan Gupta
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引用次数: 1

摘要

背景:髓外造血(EMH)是指在骨髓以外的部位,即肝脏、脾脏和淋巴结产生血液细胞成分。与这种疾病相关的常见部位是肝脏、脾脏和淋巴结,而与它相关的常见情况是骨髓纤维化、骨髓发育不良、地中海贫血、镰状细胞性贫血和真性红细胞增多症。病例描述:本报告描述了一位患有地中海贫血的年轻男性,由于胸椎椎管内病变而出现症状性脊髓压迫。手术切除并诊断为EMH病例。该男孩已完全康复,至今已有六个月无症状。结论:胸椎EMH的发生并不常见,而由此引起的有症状的脊髓压迫则更为罕见。磁共振成像(MRI)是诊断成像的首选,可提供的治疗方案包括手术减压、放射治疗、羟基脲和填充红细胞(rbc)的输血。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Extramedullary Hematopoiesis Presenting with Thoracic Spinal Cord Compression in a Young Adult with Thalassemia Major: A Case Report.

Extramedullary Hematopoiesis Presenting with Thoracic Spinal Cord Compression in a Young Adult with Thalassemia Major: A Case Report.

Extramedullary Hematopoiesis Presenting with Thoracic Spinal Cord Compression in a Young Adult with Thalassemia Major: A Case Report.

Background: Extramedullary hematopoiesis (EMH) refers to the production of blood cellular components at sites other than the bone marrow, namely liver, spleen, and lymph nodes. The common sites associated with this condition are the liver, spleen, and lymph nodes whereas the common conditions associated with it are myelofibrosis, myelodysplasia, thalassemia, sickle cell anemia, and polycythemia vera.

Case description: This report describes a young male with thalassemia major, who presented with symptomatic cord compression due to a thoracic intraspinal lesion. It was surgically excised and diagnosed as a case of EMH. The boy recovered fully and has been asymptomatic for six months now.

Conclusion: The occurrence of EMH in the thoracic spine is uncommon, whereas symptomatic cord compression as a result of it is even more unusual. Magnetic resonance imaging (MRI) is the diagnostic imaging of choice and treatment options that can be offered are surgical decompression, radiotherapy, hydroxyurea, and transfusion of packed red blood cells (RBCs).

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来源期刊
CiteScore
1.10
自引率
0.00%
发文量
49
期刊介绍: Journal of Pediatric Neurosciences-JPN (ISSN 1817-1745) is official publication of the Indian Society for Pediatric Neurosurgery. The journal is published semiannually. Bibliographic listings: The journal is indexed with Caspur, DOAJ, EBSCO Publishing’s Electronic Databases, Excerpta Medica / EMBASE, Expanded Academic ASAP, Genamics JournalSeek, Google Scholar, Health & Wellness Research Center, Health Reference Center Academic, Hinari, Index Copernicus, OpenJGate, Scimago Journal Ranking, SCOLOAR, SCOPUS, SIIC databases, Ulrich’s International Periodical Directory
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