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利用常规和游离轻链免疫固定技术检测和分析犬慢性 B 细胞淋巴细胞白血病中的副蛋白血症。

Detection and Characterization of Paraproteinemia in Canine Chronic B-cell Lymphocytic Leukemia Using Routine and Free Light Chain Immunofixation.

机构信息

Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA.

出版信息

Vet Clin Pathol. 2022 Dec;51(4):551-559. doi: 10.1111/vcp.13156. Epub 2022 Jul 26.

Abstract

BACKGROUND

Hyperglobulinemia is reported in 26% of canine chronic B-cell lymphocytic leukemia (B-CLL) cases. However, few cases have been characterized by protein electrophoresis and immunofixation (IF), and the incidence of a monoclonal protein (M-protein) is unknown using these techniques.

OBJECTIVE

To characterize and determine the proportion of canine B-CLL cases with an M-protein using plasma protein electrophoresis (PPE), routine and free light chain (fLC) IF, and to assess if productive B-CLL cases express MUM1/IRF4 by cell tube block (CTB).

METHODS

PPE, routine (targeting IgG, IgA, IgM, IgG4, and light chain) and fLC IF were performed using 48 dog B-CLL plasma samples from patients diagnosed via peripheral blood flow cytometry. CTB was performed on a separate cohort of 15 patients.

RESULTS

Hyperproteinemia (>7.5 g/dL) was present in 17/48 cases (35%). An M-protein was detected in 32/48 cases (67%). Of these, 19/32 cases (59%) had only complete (monoclonal heavy and light chain) M-proteins detected, 10/32 cases (31%) had both complete and fLC M-proteins detected, and 3/32 cases (9%) had only an fLC M-protein detected. IgM was the most common clonal immunoglobulin isotype detected (23 cases). CD21 cell counts were higher in cases with detectable M-protein. Plasma fLC IF suggested β-γ region interference, likely caused by clotting proteins. All B-CLL cases consistently expressed PAX5 and did not express MUM1/IRF4.

CONCLUSIONS

Most B-CLL cases had an M-protein and were not hyperproteinemic. Most cases with paraproteins had a complete IgM monoclonal gammopathy; a subset had documented fLCs. The prognostic significance of heavy and fLC presence should be evaluated.

摘要

背景

据报道,26%的犬慢性 B 细胞淋巴细胞白血病(B-CLL)病例存在球蛋白血症。然而,用蛋白电泳和免疫固定(IF)技术对少数病例进行了特征描述,并且这些技术对单克隆蛋白(M 蛋白)的发生率尚不清楚。

目的

通过血浆蛋白电泳(PPE)、常规和游离轻链(fLC)IF 对犬 B-CLL 病例进行特征描述并确定其 M 蛋白的比例,并评估是否表达 MUM1/IRF4 的产浆 B-CLL 病例通过细胞管块(CTB)。

方法

对 48 例经外周血流式细胞术诊断的犬 B-CLL 患者的血浆样本进行 PPE、常规(针对 IgG、IgA、IgM、IgG4 和轻链)和 fLC IF。对 15 例患者的另一个队列进行 CTB。

结果

高球蛋白血症(>7.5 g/dL)存在于 17/48 例(35%)中。在 48 例病例中有 32 例(67%)检测到 M 蛋白。其中,19/32 例(59%)仅检测到完整的(单克隆重链和轻链)M 蛋白,10/32 例(31%)同时检测到完整和 fLC M 蛋白,3/32 例(9%)仅检测到 fLC M 蛋白。最常见的克隆免疫球蛋白同种型是 IgM(23 例)。在可检测到 M 蛋白的病例中,CD21 细胞计数更高。血浆 fLC IF 提示β-γ 区干扰,可能由凝血蛋白引起。所有 B-CLL 病例均持续表达 PAX5,不表达 MUM1/IRF4。

结论

大多数 B-CLL 病例有 M 蛋白且无高球蛋白血症。大多数伴有副蛋白的病例为完整 IgM 单克隆丙种球蛋白病;一部分有记录的游离轻链。应评估重链和游离轻链存在的预后意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7d95/10087490/fe3ee36d33df/VCP-51-551-g001.jpg

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