Myelofibrosis in Western Australia: An Epidemiological Study of 29 Cases

Woodliff, H. J.; Dougan, L.

doi:10.5694/j.1326-5377.1976.tb140817.x

Cited by 12 publications

(8 citation statements)

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“…Prevalence data were reported in eight articles , with five reporting prevalence of PV ranging from 0.49 to 46.88 per 100,000. Prevalence rates for ET were reported in four studies from Europe and North America with rates ranging between 11.00 and 42.51 per 100,000.…”

Section: Resultsmentioning

confidence: 99%

“…Prevalence rates for ET were reported in four studies from Europe and North America with rates ranging between 11.00 and 42.51 per 100,000. Two studies reported PMF prevalence of 1.76 and 4.05 per 100,000. One North American study reported a prevalence rate of 93.43 per 100,000 for classic MPNs combined.…”

Section: Resultsmentioning

confidence: 99%

“…To obtain additional, relevant incidence and prevalence data that had not been included in reports, corresponding authors ( n = 12) were contacted to provide additional data. Communication was successful for 6 reports, contact details were no longer applicable for 1 corresponding author, 1 author had retired and 4 were unresponsive.…”

Section: Methodsmentioning

confidence: 99%

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How common are myeloproliferative neoplasms? A systematic review and meta‐analysis

Titmarsh¹,

Duncombe

McMullin³

et al. 2014

American J Hematol

175

121

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Myeloproliferative neoplasms (MPNs) are a heterogeneous group of diseases including polycythemia vera (PV), essential thrombocythemia (ET), and primary(idiopathic) myelofibrosis (PMF). In this systematic review, we provide a comprehensive report on the incidence and prevalence of MPNs across the globe. Electronic databases (PubMed, EMBASE, MEDLINE, and Web of Science) were searched from their inception to August 2012 for articles reporting MPN incidence or prevalence rates. A random effects meta-analysis was undertaken to produce combined incidence rates for PV, ET, and PMF. Both heterogeneity and small study bias were assessed. Thirty-four studies were included. Reported annual incidence rates ranged from 0.01 to 2.61, 0.21 to 2.27, and 0.22 to 0.99 per 100,000 for PV, ET, and PMF, respectively. The combined annual incidence rates for PV, ET, and PMF were 0.84, 1.03, and 0.47 per 100,000. There was high heterogeneity across disease entities (I 2 97.1-99.8%) and evidence of publication bias for ET and PMF (Egger test, P 5 0.007 and P 0.001, respectively).The pooled incidence reflects the rarity of MPNs. The calculated pooled incidence rates do not reflect MPN incidence across the globe due to the high unexplained heterogeneity. Improved, widespread registration of MPNs would provide better information for global comparison of the incidence and prevalence of MPNs.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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How common are myeloproliferative neoplasms? A systematic review and meta‐analysis

Titmarsh¹,

Duncombe

McMullin³

et al. 2014

American J Hematol

175

121

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“…The incidence amongst Caucasians in this population can be confidently assumed to be somewhat lower. AMM is quite rare: its incidence has been estimated at 0.5 x [15]. The probability of observing both events over a given 3-year interval time-frame is thus :…”

Section: Discussionmentioning

confidence: 99%

Sarcoidosis and agnogenic myeloid metaplasia

Reich¹

1994

Journal of Internal Medicine

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Agnogenic myeloid metaplasia (AMM) was recognized 3 years after a diagnosis of sarcoidosis in a 45-year-old man. This is the second reported instance of that association, and the 15th reported case in which systemic granulomata have been associated with a myeloproliferative disorder (MPD). In the client population at risk, this connection would be expected to occur by chance once in 1700 years. Therefore, it is unlikely that the association is fortuitous. Systemic granulomata may represent an immunologic response to antigens of neoplastic origin in MPD.

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“…The incidence of primary myelofibrosis (PMF) ranges from 0.3 to 1.5 per 100,000 [1–6], and studies [7–13] show that by the end of the second decade after diagnosis, up to 30% of patients with either polycythemia vera (PV) or essential thrombocythemia (ET) develop transformation to secondary myelofibrosis (MF).…”

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confidence: 99%

Ruxolitinib: A New JAK1/2 Inhibitor That Offers Promising Options for Treatment of Myelofibrosis

2011

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Ruxolitinib (INCB018424) is the first potent, selective, oral inhibitor of JAK1 and 2 being developed for clinical use. Its major cellular and systemic effects are proliferation inhibition, apoptosis induction and reduction in cytokine plasma levels, all mediated by the drug's inhibition of JAKs' ability to phosphorylate STAT. In initial clinical trials of its use in myelofibrosis, ruxolitinib exhibited durable efficacy in reduction of splenomegaly and alleviation of constitutional symptoms. Patients also showed weight gain and improvement in general physical condition. The dose-limiting toxicity was thrombocytopenia. In preliminary findings of a Phase III trial in patients with primary, postpolycythemia-vera, or postessential- thrombocythemia myelofibrosis, administration at an initial dosage of 15 or 20 mg twice daily led to a spleen-volume response rate (≥35% reduction at 24 weeks) of 41.9 versus 0.7% for placebo (p < 0.0001); furthermore, 45.9% of the ruxolitinib recipients had ≥50% improvement in symptom score (on the modified Myelofibrosis Symptom Assessment Form version 2.0) versus 5.3% for placebo (p < 0.0001). Ruxolitinib recipients also showed improvement in parameters of quality of life.

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Myelofibrosis in Western Australia: An Epidemiological Study of 29 Cases

Cited by 12 publications

References 0 publications

How common are myeloproliferative neoplasms? A systematic review and meta‐analysis

How common are myeloproliferative neoplasms? A systematic review and meta‐analysis

Sarcoidosis and agnogenic myeloid metaplasia

Ruxolitinib: A New JAK1/2 Inhibitor That Offers Promising Options for Treatment of Myelofibrosis

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