Thrombotic risk in secondary polycythemia resembles low-risk polycythemia vera and increases in specific subsets of patients

Krečak, Ivan; Holik, Hrvoje; Zekanović, Ivan; Perić, Martina Morić; Marketin, Tina; Coha, Božena; Gverić–Krečak, Velka; Vodanović, Marijo; Lucijanić, Marko

doi:10.1016/j.thromres.2021.11.025

Cited by 13 publications

(11 citation statements)

References 8 publications

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“…The low frequency of recurrent event among the MPN‐patients in this cohort could be explained by adequate use of aspirin, phlebotomy, and cytoreductive treatment. In other studies the thrombotic risk in SE have been diverging, some results supporting resemblance of thrombotic risk between SE and low risk PV 20 and other showing lower post‐diagnosis thrombotic rates in patients with SE compared to PV‐patients 21 …”

Section: Discussionmentioning

confidence: 84%

Erythrocytosis, thrombocytosis, and rate of recurrent thromboembolic event—A population based cohort study

Larsson

Andréasson

Holmberg

et al. 2023

European J of Haematology

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Introduction:The management to reduce risk of thromboembolic complications in polycythemia vera and essential thrombocythemia are well established, but for other conditions with elevated hemoglobin, hematocrit, or platelets there are no consensus regarding treatment and follow up.Aims: To assess frequency of elevated blood values in patients with thromboembolic event, how many of these should be investigated further regarding myeloproliferative neoplasm and if the risk of recurrent event is depending on underlying condition. Methods: Retrospective cohort study of 3931 adult patients in the county of Norrbotten, Sweden, with thromboembolism during 2017 and 2018. Results: Of the 3931 patients, 1195 had either elevated Hb, HCT, or platelets fulfilling the 2016 revised WHO criteria for PV and ET, and out of these 411 should be evaluated regarding underlying myeloproliferative neoplasms. Unexplained thrombocytosis and secondary erythrocytosis were associated with the highest rate of recurrent event as well as the most inferior restricted mean survival time. Conclusion: Elevated blood values are common in patients with thromboembolic event and the high risk of recurrent event and inferior restricted mean survival time in patients with unexplained thrombocytosis and secondary erythrocytosis implicates the importance of finding and managing the underlying condition.

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Section: Discussionmentioning

confidence: 84%

Erythrocytosis, thrombocytosis, and rate of recurrent thromboembolic event—A population based cohort study

Larsson

Andréasson

Holmberg

et al. 2023

European J of Haematology

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“…However, in this study, more than 10% of SP patients also had a history of thrombosis, which is noteworthy given the growing interest in the incidence of thrombosis in the SP population. [10][11][12] Much more research is required on the thrombosis of the SP group. When examining patients with erythrocytosis, the JAK2 V617F or exon12 test is performed after a clinical evaluation of secondary erythrocytosis.…”

Section: Discussionmentioning

confidence: 99%

Neutrophil‐to‐lymphocyte ratio and platelet‐to‐lymphocyte ratio as new possible minor criteria for diagnosis of polycythemia vera

Kim

Kwon

et al. 2023

Int J Lab Hematology

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IntroductionThe role of inflammation in the pathophysiology of polycythemia vera (PV) is important. The presence of JAK2 mutations is important in the diagnosis of PV, and serum levels of erythropoietin (EPO) also play a supporting role. However, serum EPO levels show some limitations. The neutrophil‐to‐lymphocyte ratio (NLR) and platelet‐to‐lymphocyte ratio (PLR) are a readily available marker of inflammation. Thus, we examined whether NLR & PLR might diagnose PV in erythrocytosis patients. We compared NLR & PLR and EPO diagnostic values.MethodsWe retrospectively reviewed clinical and laboratory data from two referral hospitals. Two hundred and eighty‐five patients with erythrocytosis who underwent a test for the JAK2 mutation were included. It wac classified as the PV group and the secondary polycythemia (SP) group.ResultsThe median NLR & PLR in the PV group (n = 70) was significantly higher than that in the SP group (n = 170) (NLR: 6.04 vs. 1.77, PLR: 283.18 vs. 101.56, respectively, p < 0.001). In the receiver operating characteristic analysis, the area under the curve of NLR & PLR was significantly higher than that of serum EPO (NLR vs EPO: 0.921 vs. 0.827, p = 0.003; PLR vs EPO: 0.917 vs 0.827, p = 0.003).ConclusionIn conclusion, NLR & PLR were higher in PV than in SP and showed better diagnostic value than serum EPO level, highlighting their potential as minor diagnostic criteria in patients with PV.

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“…14,47,48 Most studies have observed a lower incidence of thrombosis with secondary erythrocytosis in comparison to PV, [47][48][49] while two retrospective studies have suggested a similar thrombotic risk as in PV. 14,50 It is to be noted that the latter studies did not account for the causal diversity of secondary erythrocytosis.…”

Section: Diagnosismentioning

confidence: 99%

“…The clinical phenotype of secondary erythrocytosis differs from PV; patients with secondary erythrocytosis are more likely to be males, display isolated erythrocytosis with lower white and platelet counts, higher Epo levels, lower lactate dehydrogenase levels, without palpable splenomegaly 14,47,48 . Most studies have observed a lower incidence of thrombosis with secondary erythrocytosis in comparison to PV, 47–49 while two retrospective studies have suggested a similar thrombotic risk as in PV 14,50 . It is to be noted that the latter studies did not account for the causal diversity of secondary erythrocytosis.…”

Section: Diagnosismentioning

confidence: 99%

JAK2 unmutated erythrocytosis: 2023 Update on diagnosis and management

2023

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Disease Overview JAK2 unmutated or non‐polycythemia vera (PV) erythrocytosis encompasses a heterogenous spectrum of hereditary and acquired entities. Diagnosis Foremost in the evaluation of erythrocytosis is the exclusion of PV through JAK2 (inclusive of exons 12–15) mutation screening. Initial assessment should also include gathering of previous records on hematocrit (Hct) and hemoglobin (Hgb) levels, in order to streamline the diagnostic process by first distinguishing longstanding from acquired erythrocytosis; subsequent subcategorization is facilitated by serum erythropoietin (Epo) measurement, germline mutation screening, and review of historical data, including comorbid conditions and medication list. Hereditary erythrocytosis constitutes the main culprit in the context of longstanding erythrocytosis, especially when associated with a positive family history. In this regard, a subnormal serum Epo level suggests EPO receptor mutation. Otherwise, considerations include those associated with decreased (high oxygen affinity Hgb variants, 2,3‐bisphosphoglycerate deficiency, PIEZO1 mutations, methemoglobinemia) or normal oxygen tension at 50% Hgb saturation (P50). The latter include germline oxygen sensing pathway (HIF2A–PHD2–VHL) and other rare mutations. Acquired erythrocytosis commonly results from central (e.g., cardiopulmonary disease, high‐altitude habitat) or peripheral (e.g., renal artery stenosis) hypoxia. Other noteworthy conditions associated with acquired erythrocytosis include Epo‐producing tumors (e.g., renal cell carcinoma, cerebral hemangioblastoma) and drugs (e.g., testosterone, erythropoiesis stimulating agents, sodium‐glucose cotransporter‐2 inhibitors). Idiopathic erythrocytosis is an ill‐defined terminology that presumes the existence of an increased Hgb/Hct level without an identifiable etiology. Such classification often lacks accounting for normal outliers and is marred by truncated diagnostic evaluation. Management Current consensus treatment guidelines are not supported by hard evidence and their value is further undermined by limited phenotypic characterization and unfounded concerns for thrombosis. We are of the opinion that cytoreductive therapy and indiscriminate use of phlebotomy should be avoided in the treatment of non‐clonal erythrocytosis. However, it is reasonable to consider therapeutic phlebotomy if one were to demonstrate value in symptom control, with frequency determined by symptoms rather than Hct level. In addition, cardiovascular risk optimization and low dose aspirin is often advised. Future Directions Advances in molecular hematology might result in better characterization of “idiopathic erythrocytosis” and expansion of the repertoire for germline mutations in hereditary erythrocytosis. Prospective controlled studies are needed to clarify potential pathology from JAK2 unmutated erythrocytosis, as well as to document the therapeutic value of phlebotomy.

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Thrombotic risk in secondary polycythemia resembles low-risk polycythemia vera and increases in specific subsets of patients

Cited by 13 publications

References 8 publications

Erythrocytosis, thrombocytosis, and rate of recurrent thromboembolic event—A population based cohort study

Erythrocytosis, thrombocytosis, and rate of recurrent thromboembolic event—A population based cohort study

Neutrophil‐to‐lymphocyte ratio and platelet‐to‐lymphocyte ratio as new possible minor criteria for diagnosis of polycythemia vera

JAK2 unmutated erythrocytosis: 2023 Update on diagnosis and management

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