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Mortality outcomes and survival patterns of patients with myeloproliferative neoplasms in Malaysia

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Abstract

Background

Prognostication of myeloproliferative neoplasm (MPN) has always been challenging, even with the advent of Janus kinase 2 (JAK2 V617F) molecular studies. The survival pattern of patients diagnosed with MPN in developing countries is still undetermined.

Materials and methods

The national MPN registry conducted from 2009 to 2015 in Malaysia provided a comprehensive insight into the demographics, clinical characteristics and laboratory parameters of patients diagnosed with MPN nationwide. The study analysed the survival patterns and mortality outcomes and risk among 671 patients diagnosed with essential thrombocythaemia (ET), polycythaemia vera (PV), primary myelofibrosis (PMF) and unclassified MPN (MPN-U). Mortality status was traced and confirmed until the end of December 2018, with right censoring applied to patients alive beyond that.

Results

The analysed cohort consisted of 283 (42.2%) ET, 269 (40.1%) PV, 62 (9.2%) PMF and 57 (8.5%) MPN-U incident cases with diagnosis made between 2007 and 2015. The majority of patients were male (52.3%) and Malay (48.9%), except for ET, in which the majority of patients were female (60.1%) and of Chinese origin (47.0%). Female patients were found to have significantly better overall survival (OS) rates in ET (p = 0.0285) and MPN-U (p = 0.0070). Patients with JAK2 V617F mutation were found to have marginally inferior OS over time. Multivariable Cox regression identified patients with increased age [hazard ratio (HR) 1.055, 95% CI 1.031; 1.064], reduced haemoglobin (HB) level (HR 0.886, 95% CI 0.831; 0.945, p = 0.0002), being male (HR 1.545, 95% CI 1.077; 2.217, p = 0.0182), and having MPN-U (HR 2.383, 95% CI 1.261; 4.503, p = 0.0075) and PMF (HR 1.975, 95% CI 1.054; 3.701, p = 0.0335) at increased risk for worse mortality outcomes.

Conclusion

Myeloproliferative neoplasm reduces patient survival. The degree of impact on survival varies according to sub-type, sex, bone marrow fibrosis and HB levels. The JAK2 V617F mutation was not found to affect the survival pattern or mortality outcome significantly.

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Acknowledgments

We would like to acknowledge the Director General of the Ministry of Health, Malaysia for the approval to publish the study findings. The members of the MPN registry working group are: Sen Mui Tan, Ngee Siang Lau, Tee Chuan Ong: Ampang Hospital, Ampang, Selangor, Malaysia; Ai Sim Goh, Teng Keat Chew: Penang General Hospital, Pulau Pinang, Malaysia; Soo Min Lim: Hospital Sultanah Aminah, Johor, Malaysia; See Guan Toh: Hospital Tuanku Ja’afar, Seremban, Malaysia; Xavier Yoon Han Sim: Hospital Tengku Ampuan Rahimah, Klang, Malaysia; Padmini Menon: Hospital Raja Permaisuri Bainun, Perak, Malaysia; Yong Khee Guan: Pantai Hospital Ayer Keroh, Melaka, Malaysia; Azlan Bin Husin: Hospital Universiti Sains Malaysia, Kelantan, Malaysia; Lily Lee Lee Wong: Queen Elizabeth Hospital, Sabah, Malaysia; Lee Ping Chew: Sarawak General Hospital, Sarawak, Malaysia; Sinari Salleh: Hospital Raja Perempuan Zainab II, Kelantan, Malaysia; Kim Yen Goh: Ampang Putri Hospital, Selangor, Malaysia; Kin Wah Leong, Su Hong Lim: Gleneagles Penang Hospital, Penang, Malaysia; Syed Carlo Edmund: Institute for Clinical Research, Ministry of Health Malaysia; Jenq Tzong Tan: Taiping Hospital, Perak, Malaysia; Gin Gin Gan: University Malaya Medical Centre, Kuala Lumpur, Malaysia; Subramaniam Yegappan: Oncode Scientific Laboratory, Selangor, Malaysia; Kian Meng Chang: Sunway Medical Centre, Kuala Lumpur, Malaysia.

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Authors and Affiliations

  1. Department of Haematology, Ampang Hospital, Jalan Mewah Utara, Pandan Mewah, 68000, Ampang, Selangor, Malaysia

    Yee Yee Yap & Jameela Sathar

  2. Institute for Clinical Research, National Institutes of Health, Ministry of Health Malaysia, Kompleks Institut Kesihatan Negara, Jalan Setia Murni U13/52 Seksyen U13, Setia Alam, 40170, Shah Alam, Selangor, Malaysia

    Kian Boon Law

Authors
  1. Yee Yee Yap
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  2. Jameela Sathar
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MPN registry working group

  • Sen Mui Tan
  • , Ngee Siang Lau
  • , Tee Chuan Ong
  • , Ai Sim Goh
  • , Teng Keat Chew
  • , Soo Min Lim
  • , See Guan Toh
  • , Xavier Yoon Han Sim
  • , Padmini Menon
  • , Yong Khee Guan
  • , Azlan Bin Husin
  • , Lily Lee Lee Wong
  • , Lee Ping Chew
  • , Sinari Salleh
  • , Kim Yen Goh
  • , Kin Wah Leong
  • , Su Hong Lim
  • , Syed Carlo Edmund
  • , Jenq Tzong Tan
  • , Gin Gin Gan
  • , Subramaniam Yegappan
  •  & Kian Meng Chang

Contributions

MPN registry working group, JS and YYY contributed to data collection. JS, YYY and KBL contributed to the design of the study. YYY and KBL did the literature research. KBL performed the statistical analysis. JS, YYY and KBL contributed to the interpretation of results and manuscript preparation. All authors contributed to the manuscript editing and approval. 

Corresponding author

Correspondence to Yee Yee Yap.

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Conflict of interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Ethical approval

The registry and study received approval from the Medical Research & Ethics Committee in 2017 (registration no. NMRR-17-2250-37701).

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The members of MPN registry working group are listed in the acknowledgements section.

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Yap, Y.Y., Sathar, J., Law, K.B. et al. Mortality outcomes and survival patterns of patients with myeloproliferative neoplasms in Malaysia. Cancer Causes Control 33, 343–351 (2022). https://doi.org/10.1007/s10552-021-01521-2

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