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Serum CXCL13 level is related to treatment response and predicts disease prognosis in Waldenström macroglobulinemia

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Abstract

Waldenström macroglobulinemia (WM) is a type of B-cell lymphoma that produces IgM. Our study aimed to investigate the role of CXCL13, a chemokine essential for B lymphocytes, in the evaluation of treatment response and prognosis in WM. We collected serum samples and clinical data from 72 WM patients, with 69 patients receiving systemic therapy and 3 patients opting not to receive treatment. Serum CXCL13 levels at baseline and after six months of treatments were measured by enzyme-linked immunosorbent assay. The median serum level of CXCL13 was 1 539.2 pg/ml (range 10.0–21 389.9) at baseline and significantly decreased to 123.1 pg/ml (range 0.0–6 741.5) after 6 months of treatments. At baseline, higher CXCL13 levels were associated with lower hemoglobin levels (p = 0.001), higher β2-microglobulin levels (p = 0.001), lower albumin levels (p = 0.046), and higher IPSS-WM scores (p = 0.013). After 6 months of treatment, patients who achieved PR/VGPR had significantly lower CXCL13 levels compared to those with SD (70.2 pg/ml vs 798.6 pg/ml, p = 0.002). The median follow-up period was 40 months (range 4.2–188). Eight patients died during the follow-up period. Overall survival differed based on CXCL13 levels. When grouped by baseline CXCL13 levels, the median OS was 60.0 months in patients with serum CXCL13 > 2 000 pg/ml, while it was not reached in patients with low CXCL13 levels (p < 0.001). Based on CXCL13 levels after the treatments, the median OS was 74.0 months in patients with serum CXCL13 > 200 pg/ml, while it was not reached in patients with CXCL13 ≤ 200 pg/ml. In a subgroup of 28 patients with a series of serum samples, the increase of serum CXCL13 level was associated with disease progression or the start of next-line therapy (p < 0.001). Our study concludes that serum CXCL13 levels decrease in WM patients treated with various regimens and correlate with treatment response. Detecting serum CXCL13 at baseline or after treatment help in predicting prognosis.

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Data availability

No datasets were generated or analysed during the current study.

Abbreviations

ELISA:

Enzyme-linked immunoassay

IgM:

Immunoglobulin M

MR:

Minimal remission

OS:

Overall survival

PR:

Partial remission

SD:

Stable disease

TTNT:

Time to next treatment

VGPR:

Very good partial remission

WM:

Waldenström macroglobulinemia

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Funding

This study was supported by the National Key R&D Program of China, Ministry of Science and Technology of the People’s Republic of China (2022YFC2304605), and the National High Level Hospital Clinical Research Funding (2022-PUMCH-A-193).

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Author notes

  1. Jia Chen and Ming-nan Jia contributed equally to this study.

Authors and Affiliations

  1. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

    Jia Chen, Hao Cai, Jian Li, Dao-bin Zhou & Xin-xin Cao

  2. State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

    Jia Chen, Hao Cai, Jian Li, Dao-bin Zhou & Xin-xin Cao

  3. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

    Ming-nan Jia

  4. Department of Clinical Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

    Ze-peng Li

Authors
  1. Jia Chen
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Contributions

XXC, DBZ, and JL contributed to study conception and design; ZPL, and HC contributed to CXCL13 analysis; JC, and MNJ contributed to patient follow-up; JC, and MNJ contributed to data analysis and interpretation; JC, MNJ, and XXC retrospectively reviewed patient records and contributed to data collection; JC and MNJ wrote the paper; and all authors revised the paper and approved the submitted version.

Corresponding author

Correspondence to Xin-xin Cao.

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Ethics approval and consent to participate

In accordance with the ethical standards of the Declaration of Helsinki, informed consent was obtained from all patients, and the study was approved by the Peking Union Medical College Hospital Ethics Committee.

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Not applicable.

Competing interests

The authors declare no competing interests.

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Chen, J., Jia, Mn., Cai, H. et al. Serum CXCL13 level is related to treatment response and predicts disease prognosis in Waldenström macroglobulinemia. Ann Hematol (2024). https://doi.org/10.1007/s00277-024-05690-3

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