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再生障碍性贫血的小综述,由一例酷似骨硬化性骨转移的骨髓“热点”表现的病例报告加以说明。

A mini-review on aplastic anemia, illustrated by a case report on bone marrow hot pockets mimicking sclerotic bone metastases.

机构信息

Hematologie, UGent: Universiteit Gent, Gent, Belgium.

University Hospital Ghent: Universitair Ziekenhuis, Gent, Belgium.

出版信息

Acta Clin Belg. 2022 Apr;77(2):410-415. doi: 10.1080/17843286.2021.1872310. Epub 2021 Jan 12.

DOI:10.1080/17843286.2021.1872310
PMID:33433292
Abstract

A 61-year-old female presented with pancytopenia with a hemoglobin of 7.6 g/dL, platelet count of 26.000/µL and neutrophil count of 525/µL. Bone marrow aspirate showed moderately cellular marrow with a dysplastic erythroid lineage and poor megakaryo- and granulopoiesis without excessive blast count. Trephine biopsy revealed profoundly hypocellular marrow with rare hematopoietic elements. Conventional karyotyping was normal and next generation sequencing revealed no mutations. These findings were compatible with transfusion dependent, non-severe aplastic anaemia (AA) with grade 3 thrombopenia and neutropenia. However, diagnostic workup including CT thorax revealed unexpected sclerotic bone conversions in the spine. Additional whole body SPECT with 99mTc-HDP showed multiple bone lesions in the cervical, thoracic and lumbar spine. CT guided biopsy of D12 surprisingly revealed normal trilineage hematopoiesis. These results were very discrepant from the profoundly hypocellular marrow from the trephine biopsy. It is known that in AA residual hyperactive foci of hematopoiesis can persist; so called 'hot pockets'. MRI is the preferred imaging technique in AA; in most cases a homogeneous fatty bone marrow is found, though some patients present with a heterogeneous marrow with foci of decreased intensity, corresponding with residual foci of hematopoiesis. Imaging studies with PET-CT and PET-MRI confirm these different patterns with respectively homogeneous hypometabolism and hypometabolism with focal hyperproliferation. However, there is no previous literature on the aspect of this focal hematopoiesis on computed tomography. This is the first description of a 'hot pocket' manifesting as a sclerotic bone lesion on CT.

摘要

一位 61 岁女性因全血细胞减少症就诊,血红蛋白为 7.6g/dL,血小板计数为 26.000/µL,中性粒细胞计数为 525/µL。骨髓穿刺显示中度细胞性骨髓,红系发育不良,巨核细胞和粒细胞生成不良,无过多原始细胞计数。骨髓活检显示骨髓极度空骨,造血细胞稀少。常规核型分析正常,下一代测序未发现突变。这些发现与依赖输血的非严重再生障碍性贫血(AA)一致,伴有 3 级血小板减少和中性粒细胞减少。然而,诊断性检查包括胸部 CT 显示脊柱有意外的硬化性骨转换。全身 99mTc-HDP SPECT 显示颈椎、胸椎和腰椎多处骨病变。D12 的 CT 引导活检出人意料地显示三系造血正常。这些结果与骨髓活检的极度空骨骨髓非常不一致。众所周知,在 AA 中,残留的造血活性灶可能持续存在;所谓的“热点”。磁共振成像(MRI)是 AA 的首选影像学检查方法;大多数情况下,骨髓呈均匀脂肪化,但一些患者的骨髓呈异质性,有强度降低的病灶,对应残留的造血灶。PET-CT 和 PET-MRI 成像研究证实了这些不同的模式,分别为均匀低代谢和低代谢伴局灶性过度增殖。然而,以前没有关于 CT 上这种局灶性造血的文献。这是首次描述 CT 上的“热点”表现为硬化性骨病变。

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