Progressive multifocal leukoencephalopathy as the presenting feature in a patient with occult low-count monoclonal B-cell lymphocytosis.

Q3 Medicine

JAMMI Pub Date : 2024-03-29 eCollection Date: 2024-03-01 DOI:10.3138/jammi-2023-0021

Divya Santhanam, Stephanie Chan, Chris Nguyen, Juan Racosta, Anargyros Xenacostas, Kara Robertson, Michael Silverman

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引用次数: 0

Abstract

Introduction: Low Count Monoclonal B-Cell Lymphocytosis (LC-MBL) is a relatively poorly understood entity which has been suggested to be very common in asymptomatic adults and possibly related to infectious complications despite not progressing to CLL.

Methods: We describe the first case of Progressive Multifocal Leukoencephalopathy (PML) presenting in a 72-year-old man with LC-MBL but no other immunocompromising conditions.

Results: A diagnosis of PML was confirmed with classic MRI findings in association with a high CSF John Cunningham polyomavirus (JCV) viral load (4.09' 10⁵ copies/mL). An extensive search for underlying immunocompromising conditions only demonstrated LC-MBL representing approximately 4% of total leukocytes (0.2' 10⁹/L).

Discussion: This is the first report of PML in association with LC-MBL. Careful review of peripheral blood flow cytometry results is necessary to identify this disorder. Further study of the epidemiology and infectious complications of LC-MBL are warranted.

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以进行性多灶性白质脑病作为隐匿性低计数单克隆B细胞淋巴细胞增多症患者的表现特征。

导言：低数量单克隆B细胞淋巴细胞增多症（LC-MBL）是一种相对较少了解的疾病，有人认为它在无症状的成年人中非常常见，尽管不会发展为CLL，但可能与感染性并发症有关：我们描述了第一例进展性多灶白质脑病（PML）病例，患者是一名72岁的男性，患有LC-MBL，但没有其他免疫功能低下的病症：结果：典型的磁共振成像结果证实了PML的诊断，同时伴有高CSF约翰-坎宁安多瘤病毒（JCV）病毒载量（4.09' 105拷贝/毫升）。在对潜在的免疫功能低下情况进行广泛搜索后，仅发现 LC-MBL 约占白细胞总数的 4%（0.2' 109/L）：讨论：这是首例与 LC-MBL 相关的 PML 报告。要识别这种疾病，必须仔细审查外周血流式细胞术结果。有必要进一步研究 LC-MBL 的流行病学和感染并发症。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

JAMMI Medicine-Infectious Diseases

CiteScore

3.80

自引率

0.00%

发文量