4.6 Article

Philadelphia-negative myeloproliferative neoplasms among Kuwaiti Nationals

期刊

CANCER MEDICINE
卷 10, 期 1, 页码 365-371

出版社

WILEY
DOI: 10.1002/cam4.3633

关键词

driver mutations; epidemiology; Myeloproliferative neoplasms

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资金

  1. Kuwait Foundation for the Advancement of Sciences [P11613MM08]

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The study investigated the epidemiology of Ph-negative MPNs among Kuwaiti nationals, revealing ET as the most common subtype and JAK2V617F as the most common driver mutation. A significant percentage of patients (19.2%) presented with thrombosis at diagnosis, with males more likely to have arterial thrombosis and females more likely to have venous thrombosis.
The epidemiology, genetics, and thrombosis risk of MPNs among Arabs are largely unknown. This may be attributed to scarce epidemiological data, particularly from our region. Our study included 381 Kuwaiti nationals with Ph-negative MPNs and a confirmed driver mutation involving JAK2 (exon 12 14), CALR, or MPL. This first regional study examines the demographics, clinical parameters, and thrombosis-related attributes of the participants. This study reported a median age of 58 years, with females and males representing 54.9% and 45.1%, respectively. ET was the most frequent subtype of Ph-negative MPNs in our population, accounting for 52.0% of the cases, followed by PV, found in 34.6% of the participants, and PMF, found in 8.4% of participants. The crude annual cumulative incidence of Ph-negative MPNs in Kuwait ranged from 0.674 to 3.177 per 100,000 population across the study period. The most common driver mutation was JAK2V617F, with a frequency of 89.5%. At diagnosis, 19.2% of the patients presented with unexplained thrombosis, and almost half were of arterial origins. Males were more likely to present with arterial thrombosis than females (61.5% vs. 35.3%), whereas venous thrombotic events were more common in females than in males (47.1% vs. 17.9%; p-value = 0.025). Ph-negative MPNs in Kuwait are rare; however, thrombosis is a frequent complication, being documented in up to 19.2% of cases at presentation, more commonly at arterial sites. These findings call for thorough evaluation of patients with unexplained derangements in their hematological parameters during follow-ups.

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