Incidence and patient survival of myeloproliferative neoplasms and myelodysplastic/myeloproliferative neoplasms in the United States, 2001–12

Abstract

Summary Descriptive epidemiological information on myeloproliferative neoplasms (MPNs) and myelodysplastic (MDS)/MPNs is largely derived from single institution and European population-based studies. Data obtained following adoption of the World Health Organization classification of haematopoietic neoplasms and JAK2 V617F mutation testing are sparse. Using population-based data, we comprehensively assessed subtype-specific MPN and MDS/MPN incidence rates (IRs), IR ratios (IRRs) and relative survival (RS) in the United States (2001-2012). IRs were highest for polycythaemia vera (PV) (IR=10.9) and essential thrombocythaemia (ET) (IR=9.6). Except for ET and mastocytosis, overall IRs were significantly higher among males (IRRs=1.4-2.3). All evaluable MPNs were associated with lower IRs among Hispanic whites than non-Hispanic whites (NHWs), with the exception of BCR-ABL1-positive chronic myeloid leukaemia (CML), chronic eosinophilic leukaemia (CEL) and juvenile myelomonocytic leukaemia. Except for CEL, Asians/Pacific Islanders had significantly lower MPN IRs than NHWs. ET, MPN-unclassifiable and CEL IRs were 18%, 19% and 60% higher, respectively, among blacks than NHWs. Five-year RS was more favourable for younger (<60 years) than older individuals and for women compared with men, except for PV at older ages. RS was highest (>90%) for younger PV and ET patients and lowest (<20%) for older chronic myelomonocytic leukaemia and atypical BCR-ABL1-negative CML patients. Varying MPN and MDS/MPN incidence patterns by subtype support distinct aetiologies and/or susceptible populations. Decreased survival rates as compared to that expected in the general population were associated with every MPN subtype, highlighting the need for new treatments, particularly among older individuals.

Incidence of myeloproliferative neoplasms – trends by subgroup and age in a population‐based study in Sweden

“…The majority of studies included in the meta‐analysis were from Europe, North America and Asia. Similarly, in the study by Deadmond et al, there were no significant differences in incidence in patients of different ethnicities while Srour et al, also using the SEER register reported a lower incidence of MPNs among Hispanic Whites and in the Asians/Pacific Islanders . The Swedish Cancer Register does not hold information on race or ethnicity; however, 85–90% of the Swedish population is of Northern European descent and 10–15% of the population are first‐degree immigrants primarily from other European, Middle Eastern and North African countries.…”

A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis

“…Polycythaemia vera is uncommon in females of reproductive age, occurring in less than 0Á3 per 100 000 (Srour et al, 2016). There are only small case series describing the management of pregnancy in patients with PV (Harrison, 2005;Robinson et al, 2005;Griesshammer et al, 2008).…”

How I treat chronic myelomonocytic leukemia

“…[2][3][4][5] Median age at diagnosis is 72 years, CMML being rare in young adults. Whole genome sequencing of leukemic cell DNA identified 2 age-related molecular signatures, 6 supporting the idea that aging is the main cause of the disease.…”