毛细胞白血病（HCL）及其变异体HCLv是一种与体液免疫下降相关的B细胞恶性肿瘤。我们对目前最大规模的HCL/HCLv患者队列（n = 503）进行了COVID-19的前瞻性监测，包括症状、抗体、PCR和（或）抗原阳性。在HCL/HCLv患者中，50％（503中的253人）表现出COVID-19的迹象，其中有210人（83％）在PCR或快速抗原检测中呈阳性（HCL 238例，HCLv 15例）。在43例未见阳性检测结果的患者中，所有者均已产生抗核衣壳抗体，提示存在COVID-19暴露，其中7例未回忆起任何症状，36例有轻度症状，但未出现阳性检测结果。在210例阳性检测结果的患者中，23例、46例、129例和12例分别发生在2020年、2021年、2022年和2023年。在210例阳性患者中，175例在HCL/HCLv治疗前0.4-429个月（中位数66个月）开始治疗，132例在最后一次使用抗CD20 Mab剂量前0.2-229个月（中位数63个月）。两名患者死亡，其中包括一名年轻女性，在接种疫苗前2个月的第一线克拉布霉素后开始使用利妥昔单抗。几乎所有HCL/HCLv患者均从COVID-19中无事件地恢复，包括未接种疫苗或具有明显免疫抑制和最近治疗的患者。然而，HCL或治疗导致的正常B细胞的减少与对COVID-19的抗体反应水平降低（p = 0.0094）和恢复时间延长（p = 0.0036）相关。因此，在一个大型HCL/HCLv患者队列中，并首次确定COVID-19结果与免疫标志物之间的关系，死亡率相对较低（约为1%），后遗症不常见，恢复期在治疗后正常B细胞较低时较长。版权所有©2023年美国血液学会。

Hairy cell leukemia (HCL), like its variant HCLv, is a B-cell malignancy associated with decreased humoral immunity. We prospectively monitored the largest cohort of HCL/HCLv patients to date (n=503) for COVID-19 by symptoms, antibody, PCR and/or antigen-positivity. Fifty percent (253 of 503) of the HCL/HCLv patients (238 HCL, 15 HCLv) had evidence of COVID-19, with 210 (83%) of 253 testing positive by PCR or rapid-antigen. Of 43 without positive tests, all had nucleocapsid antibodies indicating COVID-19 exposure, 7 recalled no symptoms, and 36 had mild symptoms but without a positive test. Of the 210 testing positive, 23, 46, 129 and 12 occurred in 2020, 2021, 2022 and 2023, respectively. Of 210 testing positive, 175 began treatment for HCL/HCLv 0.4-429 (median 66) months before, and 132 had their last dose of anti-CD20 Mab 0.2-229 (median 63) months before. Two patients died, including a young woman beginning rituximab 2 months after 1st-line cladribine before vaccine availability. Nearly all HCL/HCLv patients recovered uneventfully from COVID-19 including those without vaccination or those with significant immunosuppression and recent treatment. However, decreased normal B-cells from HCL or treatment was associated with lower spike antibody levels as a response to COVID-19 (p=0.0094) and longer recovery time (p=0.0036). Thus, in a large cohort of HCL/HCLv and the first to determine relationships between COVID-19 outcome and immune markers, mortality was relatively low (~1%), sequelae were uncommon, and recovery from COVID-19 was longer if normal B-cells were low after recent treatment.Copyright © 2023 American Society of Hematology.