Incidence Rates of Myeloproliferative Neoplasms in India- A Hospital Based Study

Tuesday, 19 August 2014
Exhibit hall (Dena'ina Center)
Subhash Varma, MD , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Shano Naseem, MD , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Pankaj Malhotra, MD , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Jogeshwar Binota, MA , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Man Updesh Singh Sachdeva, MD , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Anil Sood, MA , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Neelam Varma, MD , Post-Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
INTRODUCTION:  Myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem cells characterized by proliferation of 1 or more myeloid lineages. These include BCR-ABL positive chronic myelogenous leukemia (CML), polycythemia vera (PV), primary myelofibrosis (PMF) and essential thrombocythemia (ET). Published epidemiological data regarding incidence rates for MPN are scarce for Indian population as there is no well defined cancer registry for these neoplasms and molecular diagnosis is not available to many clinicians. However, it is important to know their frequency in order to assess the burden of illness for public health planning. Also with the advent of specific targeted therapies for CML (tyrosine kinase inhibitors) and other MPN (JAK-2 inhibitors), it is even more important to know their incidences separately for future drug development.

METHODS: All cases diagnosed according to WHO criteria as MPN based upon clinical features, complete blood counts, bone marrow morphology and molecular genetic studies (for BCR-ABL fusion gene and JAK2V617F mutation) were enrolled in the study, from August 2012 to October 2013.  Following which the incidence rates of various MPN was determined.

RESULTS: During the study period, a total of 18,14,298 patients attended the out-patient department of our institute, and 231 (0.0127%) were diagnosed as MPN. Of the 231 MPN patients, 207 (89.6%) were BCR-ABL positive CML. The remaining 24 (10.4%) patients were BCR-ABL negative MPN; most common was PV diagnosed in 11 (4.7%), followed by PMF in 7 (3%) and ET in 6 (2.6%) cases. Molecular analysis of BCR-ABL negative MPN revealed JAK2V617F positivity in 9/11 (81.8%) cases of PV, 7/7 (100%) cases of PMF and 2/6 (33.3%) cases of ET.

CONCLUSIONS: We observed that incidence of BCR-ABL positive CML is much higher than other MPN, in contrast to reports from the west, where PV is the most common MPN. JAK2V617F mutational frequency in our BCR-ABL negative MPN was 81.8% for PV, 100% for PMF and 33.3% for ET, which is also different from that reported in western literature as more than 95% for PV, and ~60% for ET and PMF.

Additional research with carefully designed studies and generation of database is needed to estimate the national incidence rates of MPN. This will provide valuable information about the burden of illness to policy makers, healthcare insurers and pharmaceutical manufacturers. Because of availability of targeted therapy for MPN and their chronic disease course, it is especially important to study their epidemiology for judicious utilization of health care resources.

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