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椎旁髓外肿块:毛细胞白血病的一种不寻常表现

Extramedullary Paravertebral Mass: An Unusual Presentation of Hairy Cell Leukaemia.

作者信息

Valentini Samanidou, Aikaterini Panteli, Angelos Liontos, Dimitrios Biros, Lymperatou Diamantina, Milionis Haralampos, Eleni Kapsali

机构信息

1 Division of Internal Medicine and Infectious Diseases' Unit, University General Hospital of Ioannina, Ioannina, Greece.

Department of Haematology, University General Hospital of Ioannina, Ioannina, Greece.

出版信息

Eur J Case Rep Intern Med. 2024 Jul 26;11(8):004707. doi: 10.12890/2024_004707. eCollection 2024.

DOI:10.12890/2024_004707
PMID:39130072
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11313111/
Abstract

BACKGROUND

Hairy cell leukaemia (HCL) is an uncommon, indolent, B-cell, lymphoproliferative disorder typically involving peripheral blood, spleen and bone marrow. It is commonly presenting with pancytopenia, monocytopenia and massive splenomegaly, while accounting for 2% of lymphoid leukaemias. Cases of extranodal lesions caused by HCL are rare, although these have been reported. Here, we report a case of HCL presenting as a paravertebral mass without systemic involvement.

CASE DESCRIPTION

A 58-year-old man was admitted to our hospital due to progressive difficulty walking for a month, without any other symptoms. Blood examination noted mild anaemia with Hb=12.6 g/dl and mild thrombocytopenia of 140,000/μl. Magnetic resonance imaging (MRI) and computed tomography (CT) imaging demonstrated a T6 posterior paravertebral mass lesion, extending into the spinal canal with metastatic bone lesions along the thoracic and lumbar spine. Further imaging study with CT indicated mild splenomegaly (13.4 cm) and an enlarged abdominal lymph node (3.5 cm) near celiac trifurcation.

CONCLUSION

A core-needle biopsy from the paravertebral mass was performed. Results showed small-sized cells with round or oval nuclei, and pale cytoplasm with immunophenotype: B-cell origination with CD20+, Cyclin D1+, DBA.44+, Annexin+ and BRAF+, indicative of HCL.

LEARNING POINTS

Hairy cell leukaemia (HCL) is relatively uncommon, accounting for 2% of all leukaemia cases.Extramedullary and skeletal involvement in HCL is rare and shares morphological characteristics with other peripheral small B-cell lymphoma neoplasms.Spine biopsy is essential for diagnosis in these cases, since it is always helpful to narrow the differential diagnosis.Correct diagnosis is essential for treatment of HCL and leads most patients to clinical remission and sometimes long-term cures.

摘要

背景

毛细胞白血病(HCL)是一种罕见的、惰性的B细胞淋巴增殖性疾病,通常累及外周血、脾脏和骨髓。其常见表现为全血细胞减少、单核细胞减少和巨脾,占淋巴白血病的2%。尽管有HCL引起结外病变的病例报道,但较为罕见。在此,我们报告一例表现为椎旁肿块且无全身受累的HCL病例。

病例描述

一名58岁男性因进行性行走困难1个月入院,无其他症状。血液检查发现轻度贫血,血红蛋白(Hb)=12.6 g/dl,轻度血小板减少,为140,000/μl。磁共振成像(MRI)和计算机断层扫描(CT)显示T6椎体后缘椎旁肿块病变,延伸至椎管,并伴有胸腰椎转移性骨病变。CT进一步影像学检查显示轻度脾肿大(13.4 cm)和腹腔干分叉附近一个肿大的腹部淋巴结(3.5 cm)。

结论

对椎旁肿块进行了粗针活检。结果显示细胞体积小,核圆形或椭圆形,胞质淡染,免疫表型为:B细胞起源,CD20+、细胞周期蛋白D1+、DBA.44+、膜联蛋白+和BRAF+,提示为HCL。

学习要点

毛细胞白血病(HCL)相对罕见,占所有白血病病例的2%。HCL的髓外和骨骼受累罕见,与其他外周小B细胞淋巴瘤肿瘤具有共同的形态学特征。在这些病例中,脊柱活检对诊断至关重要,因为它有助于缩小鉴别诊断范围。正确诊断对HCL的治疗至关重要,可使大多数患者实现临床缓解,有时还能实现长期治愈。

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