Causes of erythrocytosis and its impact as a risk factor for thrombosis according to etiology: experience in a referral center in Mexico City

Olivas-Martínez, Antonio; Corona-Rodarte, Eduardo; Nuñez-Zuno, Adrián; Barrales-Benítez, Olga; Oca, Daniel Montante-Montes de; Mora, Jesús Delgado-de la; León-Aguilar, Diana; Hernández-Juárez, Hilda Elizeth; Tuna-Aguilar, Elena

doi:10.5045/br.2021.2021111

Cited by 8 publications

(6 citation statements)

References 19 publications

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“…Regarding elevated Hb/HCT, our results with one fourth of all patients with elevated Hb/HCT classified as SE is consistent with earlier studies 11,12 . In our study the participants were relatively old (median age 73.5) and the frequency of elevated Hb/HCT were 13.2%, out of these 71.6% were categorized as unexplained erythrocytosis.…”

Section: Discussionsupporting

confidence: 92%

“…In this "real-world" cohort of 3931 patients with thromboembolic event approximately one third had elevated Hb/HCT or platelet at Regarding elevated Hb/HCT, our results with one fourth of all patients with elevated Hb/HCT classified as SE is consistent with earlier studies. 11,12 In our study the participants were relatively old (median age 73.5) and the frequency of elevated Hb/HCT were 13.2%, out of these 71.6% were categorized as unexplained erythrocytosis. These results are similar to those in a study from Slovenia where 57.8% of the patients with erythrocytosis were unexplained.…”

Section: Discussionmentioning

confidence: 73%

“…The group of patients with unexplained thrombocytosis did not have the same comorbidity but had a mean age that were higher than the other subgroups. The survival of patients with erythrocytosis due to PV, obstructive sleep apnea and chronic lung disease was described in a single‐center study from Mexico, where the patients with SE had a significantly better survival than the PV‐patients 12 . These results diverge from the findings in our study where the survival of patients with SE were 2.30 years (95% CI 1.95–2.64) and MPN‐patients 2.83 years (95% CI 1.99–3.71).…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 99%

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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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“…Animal models of CSH manifest similar physiological responses [ 30 ], many of which we corroborated in this study. However, the most common form of chronic hypoxia in humans is intermittent hypoxia due to obstructive sleep apnea (OSA), which has also been reported to cause polycythemia [ 31 ] and to contribute to the development of PH and RVH through pulmonary vasoconstriction secondary to hypoxia, although other studies indicate that intermittent hypoxia is not sufficient to cause sustained PH [ 32 , 33 ].…”

Section: Discussionmentioning

confidence: 99%

Maladaptive Pulmonary Vascular Responses to Chronic Sustained and Chronic Intermittent Hypoxia in Rat

et al. 2021

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Chronic sustained hypoxia (CSH), as found in individuals living at a high altitude or in patients suffering respiratory disorders, initiates physiological adaptations such as carotid body stimulation to maintain oxygen levels, but has deleterious effects such as pulmonary hypertension (PH). Obstructive sleep apnea (OSA), a respiratory disorder of increasing prevalence, is characterized by a situation of chronic intermittent hypoxia (CIH). OSA is associated with the development of systemic hypertension and cardiovascular pathologies, due to carotid body and sympathetic overactivation. There is growing evidence that CIH can also compromise the pulmonary circulation, causing pulmonary hypertension in OSA patients and animal models. The aim of this work was to compare hemodynamics, vascular contractility, and L-arginine-NO metabolism in two models of PH in rats, associated with CSH and CIH exposure. We demonstrate that whereas CSH and CIH cause several common effects such as an increased hematocrit, weight loss, and an increase in pulmonary artery pressure (PAP), compared to CIH, CSH seems to have more of an effect on the pulmonary circulation, whereas the effects of CIH are apparently more targeted on the systemic circulation. The results suggest that the endothelial dysfunction evident in pulmonary arteries with both hypoxia protocols are not due to an increase in methylated arginines in these arteries, although an increase in plasma SDMA could contribute to the apparent loss of basal NO-dependent vasodilation and, therefore, the increase in PAP that results from CIH.

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“…Management of OSA includes weight loss and referral to a sleep specialist for institution of overnight CPAP. Regarding thrombotic risk and OSA, a recent study evaluating 330 cases of erythrocytosis found thrombotic rate to be significantly lower in patients with OSA versus those with either PV or chronic pulmonary disease (incidence rate of 1.46 vs. 4.51/6.24 cases per 100 person‐years, respectively; p = .009) 128 …”

Section: Diagnostic Approach To Acquired Erythrocytosismentioning

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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Causes of erythrocytosis and its impact as a risk factor for thrombosis according to etiology: experience in a referral center in Mexico City

Cited by 8 publications

References 19 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

Maladaptive Pulmonary Vascular Responses to Chronic Sustained and Chronic Intermittent Hypoxia in Rat

JAK2 unmutated erythrocytosis: 2023 Update on diagnosis and management

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