J Korean Med Sci.  2022 Mar;37(11):e87. 10.3346/jkms.2022.37.e87.

Volumetric Splenomegaly in Patients With Polycythemia Vera

• Lee MW
• Yeon SH
• Ryu H
• Song IC
• Lee HJ
• Yun HJ
• Kim SY
• Lee JE
• Shin KS
• Jo DY

• KMID: 2527447
• DOI: http://doi.org/10.3346/jkms.2022.37.e87

Abstract

Background
Non-palpable splenomegaly in patients with polycythemia vera (PV) has seldom been addressed. In this retrospective study, we evaluated non-palpable, volumetric splenomegaly defined based on age- and body surface area (BSA)–matched criteria in patients with PV diagnosed according to the 2016 World Health Organization diagnostic criteria.
Methods
Patients with PV who underwent abdominal computed tomography (CT) and who had palpable splenomegaly at diagnosis from January 1991 to December 2020 at Chungnam National University Hospital were enrolled. The spleen volume of each patient was determined by volumetric analysis of abdominal CT and adjusted for the patient’s age and BSA. Then the degree of splenomegaly was classified as no splenomegaly, borderline volumetric splenomegaly, overt volumetric splenomegaly, or palpable splenomegaly.
Results
Of the 87 PV patients enrolled, 15 (17.2%) had no splenomegaly, whereas 17 (19.5%), 45 (51.7%), and 10 (11.5%) had borderline volumetric, overt volumetric, and palpable splenomegaly, respectively. The degree of splenomegaly did not affect the cumulative incidence of thrombotic vascular events (10-year incidence: 7.7%, 0%, 22.3%, and 50.7%, respectively, P = 0.414). By contrast, splenomegaly tended to adversely affect myelofibrotic transformation (10-year cumulative incidence: 0%, 0%, 7.1%, and 30.3%, respectively, P = 0.062). Moreover, the cumulative incidence of myelofibrotic transformation was significantly higher in patients with overt volumetric or palpable splenomegaly than those with no or borderline volumetric splenomegaly (10-year incidence: 0% vs. 10.3%, respectively; 15-year incidence: 0% vs. 26.3%, respectively, P = 0.020). Overall survival (OS) differed among patients with different degrees of splenomegaly (15-year OS: 100%, 78.6%, 71.7%, and 51.9%, respectively, P = 0.021).
Conclusion
The degree of splenomegaly, including volumetric splenomegaly, based on ageand BSA-matched reference spleen volumes at diagnosis reflects disease progression in PV patients. Therefore, volumetric splenomegaly should be evaluated at the time of diagnosis and taken into consideration when predicting the prognosis of patients with PV.

Keyword

Polycythemia Vera; Spleen; Computed Tomography; Splenomegaly; Myelofibrosis; Survival

Figure

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  Fig. 1 Incidence of splenomegaly over time in patients with polycythemia vera.

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  Fig. 2 Cumulative incidence of myelofibrotic transformation according to the degree of splenomegaly in patients with polycythemia vera.

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  Fig. 3 Overall survival according to the degree of splenomegaly in patients with polycythemia vera.

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