Myeloproliferative neoplasms working group consensus recommendations for diagnosis and management of primary myelofibrosis, polycythemia vera, and essential thrombocythemia

MB Agarwal¹, Hemant Malhotra², Prantar Chakrabarti³, Neelam Varma⁴, Vikram Mathews⁵, Jina Bhattacharyya⁶, Tulika Seth⁷, K Gayathri⁸, Hari Menon⁹, PG Subramanian¹⁰, Ajay Sharma¹¹, Maitreyee Bhattacharyya¹², Jay Mehta¹³, AK Vaid¹⁴, Sandeep Shah¹⁵, Shyam Aggarwal¹⁶, PK Gogoi¹⁷, Reena Nair¹⁸, Usha Agarwal¹⁹, Subhash Varma²⁰, S. V. S. S. Prasad²¹, Marie Therese Manipadam^22
1 Department of Hematology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
² Division of Medical Oncology, RK Birla Cancer Center, SMS Medical College Hospital, Jaipur, Rajasthan, India
³ Department of Hematology, NRS Medical College, Kolkata, West Bengal, India
⁴ Department of Hematology and Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
⁵ Department of Hematology, Christian Medical College, Vellore, Tamil Nadu, India
⁶ Department of Clinical Hematology, Guwahati Medical College and Hospital, Assam, India
⁷ Department of Hematology, All India Institute of Medical Sciences, Delhi Cantonment, India
⁸ Department of Hematopathology, Lifeline Tapadia Diagnostic Centre, Hyderabad, Telangana, India
⁹ Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
¹⁰ Hematopathology Laboratory, Tata Memorial Hospital, Mumbai, Maharashtra, India
¹¹ Department of Hematology and Center for Stem Cell Transplantation and Research, Army Research and Referral Hospital, Delhi Cantonment, India
¹² Institute of Hematology and Transfusion Medicine, Medical College, Kolkata, West Bengal, India
¹³ Centre of Excellence in Histopathology, SRL Diagnostics, Mumbai, Maharashtra, India
¹⁴ Cancer Institute-Division of Medical Oncology and Haemotology, Medanta-The Medicity, New Delhi, India
¹⁵ Department of Medical Oncology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India
¹⁶ Department of Medical Oncology, Sir Ganga Ram Hospital, New Delhi, India
¹⁷ East India Hematological Centre, Rajgarh Road, Guwahati, Assam, India
¹⁸ Department of Clinical Hematology, Tata Medical Centre, Kolkata, West Bengal, India
¹⁹ Ashirwad Hematology Centre, Dadar, Mumbai, Maharashtra, India
²⁰ Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
²¹ Division of Medical Oncology, Apollo Cancer Hospitals, Hyderabad, Telangana, India
²² Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Hemant Malhotra
Division of Medical Oncology, RK Birla Cancer Center, SMS Medical College Hospital, Jaipur, Rajasthan
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0971-5851.151770

1. According to the 2008 revision of the World Health Organization (WHO) classification of myeloid malignancies, philadelphia chromosome (Ph)-negative myeloproliferative neoplasms (MPNs) include clonal, hematologic disorders such as polycythemia vera, primary myelofibrosis, and essential thrombocythemia.
2. Recent years have witnessed major advances in the understanding of the molecular pathophysiology of these rare subgroups of chronic, myeloproliferative disorders. Identification of somatic mutations in genes associated with pathogenesis and evolution of these myeloproliferative conditions (Janus Kinase 2; myeloproliferative leukemia virus gene; calreticulin) led to substantial changes in the international guidelines for diagnosis and treatment of Ph-negative MPN during the last few years.
3. The MPN-Working Group (MPN-WG), a panel of hematologists with expertise in MPN diagnosis and treatment from various parts of India, examined applicability of this latest clinical and scientific evidence in the context of hematology practice in India.
4. This manuscript summarizes the consensus recommendations formulated by the MPN-WG that can be followed as a guideline for management of patients with Ph-negative MPN in the context of clinical practice in India.