Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: an analysis by the MPN QOL International Working Group

Geyer, Holly; Kosiorek, Heidi E.; Dueck, Amylou C.; Scherber, Robyn M.; Slot, Stefanie; Boekhorst, Peter te; Senyak, Zhenya; Schouten, Harry C.; Sackmann, Federico; Fuentes, Ana Kerguelen; Hernández‐Maraver, Dolores; Pahl, Heike L.; Grießhammer, Martin; Stegelmann, Frank; Döhner, Konstanze; Lehmann, Thomas; Bonatz, Karin; Reiter, Andreas; Boyer, Françoise; Étienne, Gabriel; Ianotto, Jean‐Christophe; Ranta, Dana; Roy, Lydia; Cahn, Jean Yves; Harrison, Claire; Radia, Deepti; Muxí, Pablo; Maldonado, Norman; Besses, Carlos; Cervantes, Francisco; Johansson, Peter; Barbui, Tiziano; Barosi, Giovanni; Vannucchi, Alessandro M.; Paoli, Chiara; Passamonti, Francesco; Andréasson, Björn; Ferrari, Maria Luisa; Samuelsson, Jan; Cannon, Keith; Birgegård, Gunnar; Xiao, Zhijian; Xu, Zefeng; Zhang, Yue; Sun, Xiujuan; Xu, Junqiang; Kiladjian, Jean‐Jacques; Zhang, Peihong; Gale, Robert Peter; Mesa, Ruben A.

doi:10.3324/haematol.2016.149559

Cited by 52 publications

(57 citation statements)

References 41 publications

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“…QoL was significantly less disturbed in ET than in MF, also in accordance with previous reports. 21 Our findings confirm that inflammation is an important factor in the anaemia of MF and that a disturbed iron metabolism is involved The realisation that patients with cancer often have FID has been the basis for attempts to treat cancer anaemia with intravenous iron. First, it was shown that addition of iron made ESA treatment more effective and reduced the ESA doses needed for response 16,23 (Henry et al 2007).…”

Section: Discussionsupporting

confidence: 78%

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Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group

Birgegård

Samuelsson

Ahlstrand

et al. 2019

European J of Haematology

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Objectives The study investigates the hypothesis that inflammation in myelofibrosis (MF) like in myeloma and lymphoma, may disturb iron distribution and contribute to anaemia. Methods A cross‐sectional study of 80 MF and 23 ET patients was performed. Results About 35% of anaemic MF patients had functional iron deficiency (FID) with transferrin saturation <20 and normal or elevated S‐ferritin (<500 µg/L). In ET, FID was rare. In MF patients with FID, 70.6% were anaemic, vs 29.4% in patients without FID (P = 0.03). Hepcidin was significantly higher in MF patients with anaemia, including transfusion‐dependent patients, 50.6 vs 24.4 µg/L (P = 0.01). There was a significant negative correlation between Hb and inflammatory markers in all MF patients: IL‐2, IL‐6 and TNF‐α, (P < 0.01‐0.03), LD (P = 0.004) and hepcidin (P = 0.03). These correlations were also seen in the subgroup of anaemic MF patients (Table ). Tsat correlated negatively with CRP (P < 0.001). Symptom burden was heavier in MF patients with FID, and MPN‐SAF quality of life scores correlated with IL‐6 and CRP. Conclusions The inflammatory state of MF disturbs iron turnover, FID is common and contributes to anaemia development and impairment of QoL. Anaemic MF patients should be screened for FID.

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Section: Discussionsupporting

confidence: 78%

“…On the other hand, several patients had low iron stores, and one had a true iron deficiency anaemia, which is in line with the increased risk for bleeding in ET. QoL was significantly less disturbed in ET than in MF, also in accordance with previous reports …”

Section: Discussionmentioning

confidence: 99%

Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group

Birgegård

Samuelsson

Ahlstrand

et al. 2019

European J of Haematology

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“…In a study conducted in the UK and USA, significant reductions in QoL relative to controls were observed, as well as differences in QoL between UK and US patients, and in symptom burden between male and female patients [6]. This last observation was also reported in a study by the MPN QoL International Working Group [61]. The US ''Living with MPNs'' survey found that all three types of MPNs have a substantial negative impact on patients' employment status, career potential, and work productivity comparable to the impairment levels reported for other disabling chronic conditions [9,10].…”

Section: Impact Of Mpns On Patient-reported Outcomes (Pros)supporting

confidence: 54%

The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View

Petruk¹,

Mathias²

2020

Adv Ther

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“…The clinical hallmarks of both primary and secondary MF are splenomegaly, constitutional (specifically, fever, weight loss and night sweats) and/or disease-related (i.e. fatigue, pruritus and abdominal pain) symptoms, and cytopenias (mainly, anemia) [ 2 , 3 ]. MF results in severely impaired quality of life and reduced survival, particularly in patients with high and intermediate-2 risk disease according to the International Prognostic Score System (IPSS) [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Baseline factors associated with response to ruxolitinib: an independent study on 408 patients with myelofibrosis

Palandri

Palumbo²,

Bonifacio

et al. 2017

Oncotarget

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In patients with Myelofibrosis (MF) treated with ruxolitinib (RUX), the response is unpredictable at therapy start. We retrospectively evaluated the impact of clinical/laboratory factors on responses in 408 patients treated with RUX according to prescribing obligations in 18 Italian Hematology Centers. At 6 months, 114 out of 327 (34.9%) evaluable patients achieved a spleen response. By multivariable Cox proportional hazard regression model, pre-treatment factors negatively correlating with spleen response were: high/intermediate-2 IPSS risk (p=0.024), large splenomegaly (p=0.017), transfusion dependency (p=0.022), platelet count <200×109/l (p=0.028), and a time-interval between MF diagnosis and RUX start >2 years (p=0.048). Also, patients treated with higher (≥10 mg BID) average RUX doses in the first 12 weeks achieved higher response rates (p=0.019). After adjustment for IPSS risk, patients in spleen response at 6 months showed only a trend for better survival compared to non-responders. At 6 months, symptoms response was achieved by 85.5% of 344 evaluable patients; only a higher (>20) Total Symptom Score significantly correlated with lower probability of response (p<0.001). Increased disease severity, a delay in RUX start and titrated doses <10 mg BID were associated with patients achievinglower response rates. An early treatment and higher RUX doses may achieve better therapeutic results.

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Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: an analysis by the MPN QOL International Working Group

Cited by 52 publications

References 41 publications

Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group

Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group

The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View

Baseline factors associated with response to ruxolitinib: an independent study on 408 patients with myelofibrosis

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