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Volumetric splenomegaly in patients with essential thrombocythemia and prefibrotic/early primary myelofibrosis

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Abstract

Non-palpable, volumetric splenomegaly at diagnosis was evaluated using computed tomography in patients with essential thrombocythemia (ET) and prefibrotic/early primary myelofibrosis (pre-PMF) based on 2016 World Health Organization guidelines. Each patient’s spleen volume was adjusted for their age and body surface area. The degree of splenomegaly was classified as no, borderline volumetric, overt volumetric, or palpable splenomegaly. Seventy-six patients with ET (median age, 62.5 years) and 19 patients with pre-PMF (median age, 65 years) were followed up for a median of 2.4 years (range 0.1–17.6 years) and 4.2 years (range 0.2–19.6 years), respectively. Spleen volume was significantly greater in pre-PMF patients than in ET patients (377.9 ± 92.2 cm3 vs. 224.9 ± 115.2 cm3, P < 0.001). No, borderline volumetric, overt volumetric, and palpable splenomegaly were found in 42 (55.3%), 24 (31.6%), 10 (13.2%), and 0 (0%) patients with ET, respectively, and in 0 (0%), 8 (42.1%), 19 (52.6%), and 1 (5.2%) patient with pre-PMF, respectively (P < 0.001). Volumetric splenomegaly did not affect thrombosis-free survival in patients with ET or those with pre-PMF. This study indicates that all patients with pre-PMF present with splenomegaly, whereas half of the patients with ET have a normal-sized spleen at diagnosis.

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Acknowledgments

The authors thank Dr. Hiroki Shirato and his colleagues for their kind permission to access age- and body surface area-matched reference spleen volumes.

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Authors and Affiliations

  1. Division of Hematology/Oncology, Department of Internal Medicine, College of Medicine, Chungnam National University, 282 Munhwa-ro, Jung-gu, Daejeon, 35015, Korea

    Myung-Won Lee, Sang-Hoon Yeon, Hyewon Ryu, Ik-Chan Song, Hyo-Jin Lee, Hwan-Jung Yun & Deog-Yeon Jo

  2. Department of Laboratory Medicine, College of Medicine, Chungnam National University, Daejeon, Korea

    Seon Young Kim

  3. Department of Radiology, College of Medicine, Chungnam National University, Daejeon, Korea

    Jeong Eun Lee & Kyung Sook Shin

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  1. Myung-Won Lee
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Correspondence to Deog-Yeon Jo.

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Lee, MW., Yeon, SH., Ryu, H. et al. Volumetric splenomegaly in patients with essential thrombocythemia and prefibrotic/early primary myelofibrosis. Int J Hematol 114, 35–43 (2021). https://doi.org/10.1007/s12185-021-03121-x

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  • DOI: https://doi.org/10.1007/s12185-021-03121-x

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