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

Lee, Myung-Won; Yeon, Sang-Hoon; Ryu, Hyewon; Song, Ik‐Chan; Yun, Hwan‐Jung; Kim, Seon‐Young; Lee, Jeong Eun; Shin, Kyung Sook; Jo, Deog‐Yeon

doi:10.1007/s12185-021-03121-x

Cited by 6 publications

(11 citation statements)

References 22 publications

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“…This has been examined in our previous study of patients with ET and prefibrotic/early PMF (pre-PMF). 23 In that study, half of patients with ET had the normal spleen, whereas all patients with pre-PMF had borderline volumetric splenomegaly or a large spleen.…”

Section: Discussionmentioning

confidence: 80%

“…Similar findings have been reported in patients with ET and pre-PMF. 23 However, 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. Furthermore, OS worsened as the degree of splenomegaly increased.…”

Section: Discussionmentioning

confidence: 90%

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Volumetric Splenomegaly in Patients With Polycythemia Vera

et al. 2022

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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 age- and 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.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 90%

Volumetric Splenomegaly in Patients With Polycythemia Vera

et al. 2022

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“…However, asymptomatic splanchnic thrombosis is sometimes encountered in clinical practice ( 24 ). Furthermore, we observed that not only palpable but also volumetric splenomegaly (as revealed by CT) had certain clinical implications ( 8 ). Overall, the clinical utility of abdominal CT at the MPN diagnosis remains unclear.…”

Section: Discussionmentioning

confidence: 98%

“…Splenomegaly was defined using previously described criteria ( 8 ). In brief, “palpable splenomegaly” indicated that the spleen was palpable below the left costal margin, and “volumetric splenomegaly” indicated that the spleen volume was greater than the mean plus three standard deviations of reference volumes based on both age and body surface area.…”

Section: Methodsmentioning

confidence: 99%

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Splenic Infarction in Patients with Philadelphia-negative Myeloproliferative Neoplasms

et al. 2022

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Objective We retrospectively analyzed the prevalence and clinical features of splenic infarctions in patients with Philadelphia chromosome-negative myeloproliferative neoplasms (Ph -MPNs). Patients Patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV), prefibrotic/early primary myelofibrosis (pre-PMF), or PMF from January 1996 to October 2020 in Chungnam National University Hospital, Daejeon, Korea, were reviewed. Results A total of 347 patients (143 ET, 129 PV, 44 pre-PMF, and 31 PMF patients; 201 men and 146 women) with a median age of 64 (range 15-91) years old were followed up for a median of 4.7 (range 0.1-26.5) years. Fifteen (4.3%) patients exhibited splenic infarctions at the diagnosis. These were most common in PMF patients (12.9%), followed by pre-PMF (9.1%) and PV (5.4%) patients. Multifocal infarcts (60.0%) were most common, followed by solitary (33.3%) and extensive infarcts (6.7%). The cumulative incidence of thrombosis in patients with splenic infarctions tended to be higher than in those lacking infarctions (10-year incidence 46.7% vs. 21.0% in PV; p=0.215; 33.3% vs. 17.9% in pre-PMF; p=0.473) patients, but statistical significance was lacking. Palpable splenomegaly (hazard ratio 14.89; 95% confidence interval 4.00-55.35; p< 0.001) was the only independent risk factor for splenic infarction. During follow-up, 5 (1.4%) patients developed splenic infarctions. Conservative treatment adequately controlled the symptoms; no serious complications were noted in any patient. Conclusion Splenic infarctions occurred most frequently in patients with PMF; it was rare in patients with ET. The clinical courses were generally mild.

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A lasso and random forest model using flow cytometry data identifies primary myelofibrosis

Zhang,

Wang,

Chang

et al. 2024

Cytometry Part B Clinical

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Thrombocythemia (ET), polycythemia vera (PV), primary myelofibrosis (PMF), prefibrotic/early (pre‐PMF), and overt fibrotic PMF (overt PMF) are classical Philadelphia‐Negative (Ph‐negative) myeloproliferative neoplasms (MPNs). Differentiating between these types based on morphology and molecular markers is challenging. This study aims to clarify the application of flow cytometry in the diagnosis and differential diagnosis of classical MPNs. This study retrospectively analyzed the immunophenotypes, clinical characteristics, and laboratory findings of 211 Ph‐negative MPN patients, including ET, PV, pre‐PMF, overt PMF, and 47 controls. Compared to ET and PV, PMF differed in white blood cells, hemoglobin, blast cells in the peripheral blood, abnormal karyotype, and WT1 gene expression. PMF also differed from controls in CD34+ cells, granulocyte phenotype, monocyte phenotype, percentage of plasma cells, and dendritic cells. Notably, the PMF group had a significantly lower plasma cell percentage compared with other groups. A lasso and random forest model select five variables (CD34+CD19+cells and CD34+CD38− cells on CD34+cells, CD13dim+CD11b− cells in granulocytes, CD38str+CD19+/−plasma, and CD123+HLA‐DR−basophils), which identify PMF with a sensitivity and specificity of 90%. Simultaneously, a classification and regression tree model was constructed using the percentage of CD34+CD38− on CD34+ cells and platelet counts to distinguish between ET and pre‐PMF, with accuracies of 94.3% and 83.9%, respectively. Flow immunophenotyping aids in diagnosing PMF and differentiating between ET and PV. It also helps distinguish pre‐PMF from ET and guides treatment decisions.

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

Cited by 6 publications

References 22 publications

Volumetric Splenomegaly in Patients With Polycythemia Vera

Volumetric Splenomegaly in Patients With Polycythemia Vera

Splenic Infarction in Patients with Philadelphia-negative Myeloproliferative Neoplasms

A lasso and random forest model using flow cytometry data identifies primary myelofibrosis

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