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CHRONIC MYELOPROLIFERATIVE NEOPLASMS

Splenomegaly (SPML) in polycythemia vera (PV): its clinical significance and its relation to symptoms, post-polycythemic myelofibrosis (PPMF) and survival

Leukemia volume 37, pages 691–694 (2023)Cite this article

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Splenomegaly (SPML) in PV is important because it has been considered a diagnostic criterion [1], yet its frequency reported in the literature ranges from 7 to 90% [1, 2]. Its presence influences therapy decisions [3, 4] and the design of clinical trials [5]. Significant SPML in newly diagnosed PV may signal disease evolution to PPMF [6]. Therapeutically, this is relevant, because interferon may be effective in reversing fibrosis [7, 8]. Since review of published literature related to these issues revealed a paucity of data and variable findings we therefore studied SPML in our PV patients at the time of diagnosis (DX) or presentation (PRES). We determined its frequency and correlated this finding with other parameters of disease, including symptoms, thrombosis, development of PPMF, and survival. As a tertiary referral center, the distinction between DX and PRES is important, since it could account for some spleen size variation previously reported in the literature.

This single-center retrospective study was approved by the WCM institutional review board. We reviewed PV records in our research data repository based on an automated query system which aggregated longitudinal clinical information for analysis of spleen size according to methods previously described [8]. Standardized diagnostic criteria for PV were used for all pts [9]. Measured spleen size on physical exam was recorded in centimeters (cm) in the midclavicular line below the left costal margin. Degree of SPML was categorized into 3 subgroups: (1) “No SPML,” spleen edge not palpable or “thought” to be palpable <1 cm; (2) palpable 1–5 cm; and (3) palpable ≥5 cm. If an ultrasound had been performed, the spleen size was similarly categorized based upon the conversion method and verified formula of Chow, et al. [10].

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Fig. 1: Overall survival (OS) and myelofibrosis-free survival (MFS) by splenomegaly group.

Data availability

The data sets generated from our automated data repository are not publicly available since they are involved in active research. All data generated and analyzed are included in the published article.

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Acknowledgements

Funded by the Johns Family Fund of the Cancer Research and Treatment Fund (CR&T), New York, NY, USA. We thank Ms. Mara Sanderson for technical assistance. Presented in part at the 63rd Annual Meeting of the American Society of Hematology, 10 Dec 2021, Atlanta, Georgia, USA.

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  1. Richard T Silver, MD Myeloproliferative Neoplasms Center, Division of Hematology-Oncology, Weill Cornell Medicine, New York, NY, USA

    Richard T. Silver, Katie Erdos, Elwood Taylor III, Joseph M. Scandura & Ghaith Abu-Zeinah

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Contributions

RTS: Designed study, analyzed data and graphs, wrote the initial paper and final paper. KE: Retrieved and analyzed data, performed statistical analysis, reviewed drafts, wrote the final paper. ET: Retrieved and analyzed data, performed statistical analysis. GAZ: Reviewed data, performed statistical analysis, reviewed drafts, wrote the final paper. JS: Supervised statistical plan and reviewed drafts. All authors reviewed final document.

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Correspondence to Richard T. Silver.

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RTS: Chair, Data Safety Monitoring Board, PharmaEssentia Corp. KE, ET, JS, GAZ: Nothing to disclose.

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Silver, R.T., Erdos, K., Taylor, E. et al. Splenomegaly (SPML) in polycythemia vera (PV): its clinical significance and its relation to symptoms, post-polycythemic myelofibrosis (PPMF) and survival. Leukemia 37, 691–694 (2023). https://doi.org/10.1038/s41375-022-01793-w

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