Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms: A Population-based Study

Montani, David; Thoré, Pierre; Mignard, Xavier; Jaïs, Xavier; Boucly, Athénaïs; Jevnikar, Mitja; Seferian, Andrei; Jutant, Étienne-Marie; Cottin, Vincent; Fadel, Élie; Simonneau, Gérald; Savale, Laurent; Sitbon, Olivier; Humbert, Marc

doi:10.1164/rccm.202210-1941oc

Cited by 7 publications

(7 citation statements)

References 61 publications

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“…Moreover, recent discoveries disclose a higher prevalence of JAK2 V617F-positive CHIP in precapillary pulmonary hypertension (PH) patients compared to healthy subjects. Mice models showed that JAK2 V617F-induced STAT3 phosphorylation further upregulates ALK1-Smad1/5/8, inducing PH and arterial remodeling in mice [71,72].…”

Section: Comprehensive Genomic Profiling Of Thrombosis Risk 231 Other...mentioning

confidence: 99%

Exploring the Molecular Aspects of Myeloproliferative Neoplasms Associated with Unusual Site Vein Thrombosis: Review of the Literature and Latest Insights

Morsia,

Torre,

Martini

et al. 2024

IJMS

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Myeloproliferative neoplasms (MPNs) are the leading causes of unusual site thrombosis, affecting nearly 40% of individuals with conditions like Budd–Chiari syndrome or portal vein thrombosis. Diagnosing MPNs in these cases is challenging because common indicators, such as spleen enlargement and elevated blood cell counts, can be obscured by portal hypertension or bleeding issues. Recent advancements in diagnostic tools have enhanced the accuracy of MPN diagnosis and classification. While bone marrow biopsies remain significant diagnostic criteria, molecular markers now play a pivotal role in both diagnosis and prognosis assessment. Hence, it is essential to initiate the diagnostic process for splanchnic vein thrombosis with a JAK2 V617F mutation screening, but a comprehensive approach is necessary. A multidisciplinary strategy is vital to accurately determine the specific subtype of MPNs, recommend additional tests, and propose the most effective treatment plan. Establishing specialized care pathways for patients with splanchnic vein thrombosis and underlying MPNs is crucial to tailor management approaches that reduce the risk of hematological outcomes and hepatic complications.

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Section: Comprehensive Genomic Profiling Of Thrombosis Risk 231 Other...mentioning

confidence: 99%

Exploring the Molecular Aspects of Myeloproliferative Neoplasms Associated with Unusual Site Vein Thrombosis: Review of the Literature and Latest Insights

Morsia,

Torre,

Martini

et al. 2024

IJMS

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“…Group 5 PH has been described among patients with myeloproliferative neoplasms (MPN). Among patients with MPNs and pre-capillary PH, CTEPH and group 5 PH were the most common etiologies of PH [ 14 ]. Other rare etiologies of PH in patients with cancer include pulmonary tumor thrombotic microangiopathy (PTTM) and PVOD, which are almost always fatal [ 31 , 32 , 33 ].…”

Section: Pulmonary Hypertension In Cancermentioning

confidence: 99%

“…Additionally, cancer is a prothrombotic state and is associated with an increased risk of venous thromboembolism (VTE) including pulmonary embolism and potentially the development of chronic thromboembolic pulmonary hypertension (CTEPH) in these patients [ 13 ]. Certain types of malignancies may be associated with the development of PH themselves, including myeloproliferative neoplasms (group 5) [ 14 , 15 ]. We have reviewed mechanisms, diagnostic approach, and management for cancer therapy-related PH and RV dysfunction.…”

Section: Introductionmentioning

confidence: 99%

Cancer Therapy-Associated Pulmonary Hypertension and Right Ventricular Dysfunction: Etiologies and Prognostic Implications

Leiva,

Beaty,

Soo

et al. 2024

Rev. Cardiovasc. Med.

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Advances in cancer therapies have improved oncologic outcomes but can potentially expose patients to risk of cardiovascular toxicity. While left ventricular (LV) dysfunction is a well-known cardiotoxicity of cancer therapy. Pulmonary hypertension (PH) and right ventricular (RV) dysfunction are seen with several cancer therapies, including alkylating agents, tyrosine kinase inhibitors (TKIs), and immunotherapy, and are associated with significant morbidity and mortality. Awareness and recognition of cancer therapy-associated PH and RV dysfunction is critical to identify underlying etiologies and institute the appropriate therapy. However, gaps exist in the current literature on the epidemiology of PH and RV dysfunction in cancer, underlying pathophysiology and optimal management strategies.

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“…Recently, increasing evidence supports the involvement of hematological disorders, especially myeloproliferative neoplasms (MPN), in the pathogenesis of CTEPH. Such evidence includes the higher rates of thrombotic events and recurrent venous thromboembolism in patients with MPN, 11,12 the elevated prevalence of CTEPH in patients with MPN, 13,14 and increased mortality after pulmonary endarterectomy in patients with CTEPH complicated with MPN. 15 Clonal hematopoiesis of indeterminate potential (CHIP), defined as the presence of an expanded somatic blood cell clone in individuals without a known hematological cancer or other clonal states, is an early stage of MPN and hematological cancers.…”

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confidence: 99%

Clonal Hematopoiesis of Indeterminate Potential in Chronic Thromboembolic Pulmonary Hypertension: A Multicenter Study

Liu,

Zhou,

Lian

et al. 2024

Hypertension

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BACKGROUND: The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is multifactorial and growing evidence has indicated that hematological disorders are involved. Clonal hematopoiesis of indeterminate potential (CHIP) has recently been associated with an increased risk of both hematological malignancies and cardiovascular diseases. However, the prevalence and clinical relevance of CHIP in patients with CTEPH remains unclear. METHODS: Using stepwise calling on next-generation sequencing data from 499 patients with CTEPH referred to 3 centers between October 2006 and December 2021, CHIP mutations were identified. We associated CHIP with all-cause mortality in patients with CTEPH. To provide insights into potential mechanisms, the associations between CHIP and inflammatory markers were also determined. RESULTS: In total, 47 (9.4%) patients with CTEPH carried at least 1 CHIP mutation at a variant allele frequency of ≥2%. The most common mutations were in DNMT3A , TET2 , RUNX1 , and ASXL1 . During follow-up (mean, 55 months), deaths occurred in 22 (46.8%) and 104 (23.0%) patients in the CHIP and non-CHIP groups, respectively ( P <0.001, log-rank test). The association of CHIP with mortality remained robust in the fully adjusted model (hazard ratio, 2.190 [95% CI, 1.257–3.816]; P =0.006). Moreover, patients with CHIP mutations showed higher circulating interleukin-1β and interleukin-6 and lower interleukin-4 and IgG galactosylation levels. CONCLUSIONS: This is the first study to show that CHIP mutations occurred in 9.4% of patients with CTEPH are associated with a severe inflammatory state and confer a poorer prognosis in long-term follow-up.

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Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms: A Population-based Study

Cited by 7 publications

References 61 publications

Exploring the Molecular Aspects of Myeloproliferative Neoplasms Associated with Unusual Site Vein Thrombosis: Review of the Literature and Latest Insights

Exploring the Molecular Aspects of Myeloproliferative Neoplasms Associated with Unusual Site Vein Thrombosis: Review of the Literature and Latest Insights

Cancer Therapy-Associated Pulmonary Hypertension and Right Ventricular Dysfunction: Etiologies and Prognostic Implications

Clonal Hematopoiesis of Indeterminate Potential in Chronic Thromboembolic Pulmonary Hypertension: A Multicenter Study

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