Divya Santhanam, Stephanie Chan, Chris Nguyen, Juan Racosta, Anargyros Xenacostas, Kara Robertson, Michael Silverman
{"title":"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。","authors":"Divya Santhanam, Stephanie Chan, Chris Nguyen, Juan Racosta, Anargyros Xenacostas, Kara Robertson, Michael Silverman","doi":"10.3138/jammi-2023-0021","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>A diagnosis of PML was confirmed with classic MRI findings in association with a high CSF John Cunningham polyomavirus (JCV) viral load (4.09' 10<sup>5</sup> copies/mL). An extensive search for underlying immunocompromising conditions only demonstrated LC-MBL representing approximately 4% of total leukocytes (0.2' 10<sup>9</sup>/L).</p><p><strong>Discussion: </strong>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.</p>","PeriodicalId":36782,"journal":{"name":"JAMMI","volume":"9 1","pages":"52-56"},"PeriodicalIF":0.0000,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10984313/pdf/","citationCount":"0","resultStr":"{\"title\":\"Progressive multifocal leukoencephalopathy as the presenting feature in a patient with occult low-count monoclonal B-cell lymphocytosis.\",\"authors\":\"Divya Santhanam, Stephanie Chan, Chris Nguyen, Juan Racosta, Anargyros Xenacostas, Kara Robertson, Michael Silverman\",\"doi\":\"10.3138/jammi-2023-0021\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>A diagnosis of PML was confirmed with classic MRI findings in association with a high CSF John Cunningham polyomavirus (JCV) viral load (4.09' 10<sup>5</sup> copies/mL). An extensive search for underlying immunocompromising conditions only demonstrated LC-MBL representing approximately 4% of total leukocytes (0.2' 10<sup>9</sup>/L).</p><p><strong>Discussion: </strong>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.</p>\",\"PeriodicalId\":36782,\"journal\":{\"name\":\"JAMMI\",\"volume\":\"9 1\",\"pages\":\"52-56\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-03-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10984313/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"JAMMI\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.3138/jammi-2023-0021\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/3/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"JAMMI","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.3138/jammi-2023-0021","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/3/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Progressive multifocal leukoencephalopathy as the presenting feature in a patient with occult low-count monoclonal B-cell lymphocytosis.
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.