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一例罕见的B细胞幼淋巴细胞白血病:佐治亚州首例确诊病例及文献综述

A Rare Case of B-cell Prolymphocytic Leukemia: The First Documented Diagnosis in Georgia With a Comprehensive Literature Review.

作者信息

Maisuradze Giorgi, Akhvlediani Giorgi, Dzodzuashvili Elene, Gersamia Tamar, Sturua Maia

机构信息

Biomedical Sciences, Georgian-American University, Tbilisi, GEO.

Internal Medicine, Ilia State University, Tbilisi, GEO.

出版信息

Cureus. 2025 Jan 2;17(1):e76787. doi: 10.7759/cureus.76787. eCollection 2025 Jan.

DOI:10.7759/cureus.76787
PMID:39897243
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11786526/
Abstract

B-cell prolymphocytic leukemia (B-PLL) is a rare and aggressive malignancy of mature B-cells associated with poor prognosis, limited treatment options, and a median survival of approximately three years. We report the first documented case of B-PLL in the country of Georgia, involving a 64-year-old male presenting with fever, night sweats, weight loss, and generalized lymphadenopathy. Initial investigations revealed significant lymphocytosis and a predominance of prolymphocytes on the peripheral smear. The diagnosis was confirmed through bone marrow aspirate analysis and flow cytometry, demonstrating clonal B-cell proliferation with a characteristic immunophenotype. The patient was treated with a rituximab-bendamustine (R-B) regimen, achieving complete remission with no detectable minimal residual disease. Remarkably, he remains in clinical remission two years post-diagnosis, underscoring the potential of early and precise diagnosis combined with individualized therapeutic strategies in managing B-PLL. This case highlights the importance of further research to optimize treatment approaches and improve outcomes in this rare and challenging disease.

摘要

B细胞幼淋巴细胞白血病(B-PLL)是一种罕见的侵袭性成熟B细胞恶性肿瘤,预后较差,治疗选择有限,中位生存期约为三年。我们报告了格鲁吉亚首例有记录的B-PLL病例,患者为一名64岁男性,表现为发热、盗汗、体重减轻和全身淋巴结肿大。初步检查发现淋巴细胞显著增多,外周血涂片显示幼淋巴细胞占优势。通过骨髓穿刺分析和流式细胞术确诊,显示为具有特征性免疫表型的克隆性B细胞增殖。患者接受了利妥昔单抗-苯达莫司汀(R-B)方案治疗,达到完全缓解,无可检测到的微小残留病。值得注意的是,诊断后两年他仍处于临床缓解状态,这突出了早期精确诊断与个体化治疗策略相结合在B-PLL治疗中的潜力。该病例强调了进一步研究以优化治疗方法并改善这种罕见且具有挑战性疾病的治疗结果的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/b566e49c0d14/cureus-0017-00000076787-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/87245485d0ed/cureus-0017-00000076787-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/b89093379308/cureus-0017-00000076787-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/e4581708ab41/cureus-0017-00000076787-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/b566e49c0d14/cureus-0017-00000076787-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/87245485d0ed/cureus-0017-00000076787-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/b89093379308/cureus-0017-00000076787-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/e4581708ab41/cureus-0017-00000076787-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3488/11786526/b566e49c0d14/cureus-0017-00000076787-i04.jpg

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本文引用的文献

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2
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J Hematol. 2023 Apr;12(2):82-86. doi: 10.14740/jh1096. Epub 2023 Apr 30.
3
De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase.
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4
Chronic lymphocytic leukaemia and prolymphocytic leukaemia. Two coins or two sides of the same coin?慢性淋巴细胞白血病和幼淋巴细胞白血病。是两枚硬币还是同一枚硬币的两面?
Haematologica. 2020 Sep 1;105(9):e484. doi: 10.3324/haematol.2020.253062.
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