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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2018; 39(04): 503-515
DOI: 10.4103/ijmpo.ijmpo_88_17
Review Article

Revised Myeloproliferative Neoplasms Working Group Consensus Recommendations for Diagnosis and Management of Primary Myelofibrosis, Polycythemia Vera, and Essential Thrombocythemia

Mohan B Agarwal
Department of Hematology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra
,
Hemant Malhotra
Division of Medical Oncology, RK Birla Cancer Center, SMS Medical College Hospital, Jaipur, Rajasthan
,
Prantar Chakarborti
Department of Hematology, NRS Medical College, Kolkata, West Bengal
,
Neelam Varma
Departments of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh
,
Vikram Mathews
Department of Hematology, Christian Medical College, Vellore, Tamil Nadu
,
Jina Bhattacharyya
Department of Clinical Hematology, Gauhati Medical College and Hospital, Guwahati, Assam
,
Tulika Seth
Department of Hematology, All India Institute of Medical Sciences, New Delhi
,
K Gyathri
Department of Hematopathology, Lifeline Tapadia Diagnostic Centre, Hyderabad, Telangana
,
Hari Menon
Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra
,
P G Subramanian
Division of Medical Oncology, Apollo Cancer Hospitals, Hyderabad, Telangana
,
Ajay K Sharma
Department of Hematology, Center for Stem Cell Transplantation and Research, Army Research and Referral Hospital, New Delhi
,
Maitreyee Bhattacharyya
Department of Hematology, Institute of Hematology and Transfusion Medicine, Medical College, Kolkata, West Bengal
,
Jay Mehta
Department of Centre of Excellence in Histopathology, SRL Diagnostics, Mumbai, Maharashtra
,
Sandeep Shah
Department of Medical Oncology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India
,
P K Gogoi
East India Hematological Centre, Guwahati, Assam
,
Reena Nair
Department of Clinical Hematology, Tata Medical Centre, Kolkata, West Bengal
,
Usha Agarwal
Ashirwad Hematology Centre, Mumbai, Maharashtra
,
Subhash Varma
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh
,
S V V S Prasad
Division of Medical Oncology, Apollo Cancer Hospitals, Hyderabad, Telangana
,
Deepak K Mishra
Division of Laboratory Medicine, Tata Medical Center, Kolkata, West Bengal
› Author Affiliations Financial support and sponsorship Nil.
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Abstract

Myeloproliferative neoplasms (MPNs) are clonal disorders, derived from abnormal hematopoietic stem cells and result in an excessive production of blood cells. This MPN group of conditions encompasses different diseases with overlapping clinical and biologic similarities. The majority of the conventional therapies of MPN are palliative in nature. However, with the discovery of Janus Kinase 2 (JAK2) mutation and development of targeted JAK1/2 inhibition therapy, the therapeutic options in treatment landscape have changed dramatically. This article presents the revised Indian MPNs Working Group consensus recommendations. It highlights and brings into attention about the recent findings that have defined the state of the art of the diagnosis and therapy in the MPN area, including identification of the new driver and prognostic mutations, treatment goals in the management of myelofibrosis and polycythemia vera (PV), role of the recently approved, targeted tyrosine kinase inhibitor ruxolitinib in PV, and special issues such MPN consideration in patients with splenic vein thrombosis and the management of the disease in pregnancy.

Keywords

Essential thrombocythemia Janus kinase - myelofibrosis - myeloproliferative neoplasm - polycythemia vera


Publication History

Article published online:
17 June 2021

© 2018. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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