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. 2022 Dec 8:13:1069360.
doi: 10.3389/fimmu.2022.1069360. eCollection 2022.

Prognosis of hyperviscosity syndrome in newly diagnosed multiple myeloma in modern-era therapy: A real-life study

Pierre-Edouard Debureaux  1 , Stéphanie Harel  1 , Nathalie Parquet  2 , Virginie Lemiale  3 , Virginie Siguret  4 , Laurie Goubeau  4 , Florence Morin  5 , Bruno Royer  1 , Wendy Cuccuini  6 , Dikelele Elessa  1 , Floriane Theves  1 , Anne C Brignier  2 , Elie Azoulay  3   7 , Bertrand Arnulf  1   7 , Alexis Talbot  1   7
Affiliations

Prognosis of hyperviscosity syndrome in newly diagnosed multiple myeloma in modern-era therapy: A real-life study

Pierre-Edouard Debureaux et al. Front Immunol. .

Abstract

Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden. Studies about the prognosis of HVS in modern-era therapy for NDMM are missing. We investigated a retrospective cohort study of NDMM with HVS between 2011-2021. Thirty-nine NDMM patients with HVS were included. HVS presentation was heterogeneous, with asymptomatic, mild, and neurological forms in 23%, 59%, and 18% of cases, respectively. No thrombosis or major bleeding was observed. Therapeutic plasma exchanges were used in 92% of patients, which were effective and well tolerated. No rebound effect was observed. All patients except one had at least one CRAB criterion. Most of the patients received bortezomib and high-dose steroids (95%) associated with an immunomodulatory drug (43%) or alkylating agents (42%). HVS in NDMM patients had dismal overall survival matched to multiple myeloma patient controls (without HVS) in our center (median: 3.6 vs. 7.7 years, p=0.01), as confirmed by multivariate analysis. Early deaths (in the first two months) occurred in 21% of older patients (>65 years). HVS in NDMM patients is a rare but life-threatening complication associated with high lethality in older patients and be a potential dismal prognosis factor in the modern treatment era.

Keywords: HVS; TPE; myeloma; neurological; prognosis.

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Conflict of interest statement

AB received support from OCTAPHARMA for attending a meeting. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Flowchart of the study.
Figure 2
Figure 2
Description and outcomes of HVS NDMM (A) Monoclonal protein level between Ig type and HVS symptoms for NDMM. Red stars denote severe cases with neurological symptoms. (B–F) Survival of HVS NDMM patients. Time to next treatment (B) and overall survival (C) of NDMM with HVS. (D–F) Overall survival based on presence of plasma cells in blood by cytology analysis (D), score R-ISS (low=R-ISS1 and R-ISS2; high=R-ISS3) (E) and cytogenetic risk (high risk= presence of t(4;14) or del(17p) by FISH; standard risk=others) (F). Number of patients could vary related to missing data. NS, not significant.
Figure 3
Figure 3
Comparison to NDMM controls. (A) Monoclonal protein level between HVS and controls for each sub-type (B, C) PT expressed in INR (B) and fibrinogen (C) levels between HVS and control NDMM. Line represents in each figure the cut-off of increase bleeding risk in clinical (D) OS of HVS (red) and controls (blue) patients, (E) Forest plot for OS with final model. In univariate, HVS, cytogenetic risk, ISS and R-ISS have a p < 0.1. Each patient with NDMM and HVS was paired with two NDMM controls without HVS based on sex, age ( ± 5 years), year of diagnosis ( ± 3 years), and first-line MM therapy. The median follow-up for the controls (n=76) was five years (IQR 2.7–8).
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