A review of second malignancies in Philadelphia (Ph.) chromosome negative myeloproliferative disorders

Keywords: 2nd malignnacies, MPN, Therapy, Surveilllance

Abstract

As the survival of cancers is improving, second malignancies have emerged as a major cause of morbidity and mortality in many cancers with the overall incidence of secondary cancers at 8%. More than 50% of such patients with secondary cancers will die due to their second malignancy. Second cancers are particularly common in lymphoid malignancies but are also seen in myeloid malignancies.1,2 In haematological malignancies, advances in molecular biology, modern diagnostics, stem cell transplantation and evolution of clinical trials in which multi agent chemotherapy protocols and new targeted drugs are being tested has allowed survival to gradually improve over the last several decades.3,4 However, autologous transplant and previous chemotherapy (particularly alkylating agents and purine analogues are implicated in inducing secondary genetic abnormalities and subsequent development of secondary myelodysplasia and acute leukaemia.5,6,7 Spontaneous malignancies are also noted after long-term lenalidomide therapy in multiple myeloma 8,9 and, more recently, after Chimeric Antigen Receptor (CAR-T) T-cell therapy with a varied incidence of 4-16%.10,11,12

Ph. negative myeloproliferative disorders (MPNs) have a relatively long survival, some like Polycythaemia Vera (PV) have a median five year survival of 85% which is equivalent to closely matched controls and, similarly, in Essential Thrombocytosis (ET) the 5 year survival is equivalent to matched healthy people.13 In MPNs particularly PV, vascular events and transformation to myelofibrosis and acute leukaemia are a major cause of mortality.14,15,16 Recently however second malignancies have been recognised as an additional risk factor for mortality in these disorders.17

This review is aimed at raising awareness amongst general physicians regarding the risk of second malignancies in Ph.-negative MPNs and the need for vigilance, screening and continued research.

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Author Biography

Farooq Ahmad Wandroo, Sandwell and West Birmingham Hospitals NHS Trust, University of Birmingham,UK

Heaf of Clinical Haematology,  Honorary Associate Professor

Deapartment of Clinical and Laboratory Haematology

SWBH NHS Trust

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Published
2024-09-27
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Wandroo F. A review of second malignancies in Philadelphia (Ph.) chromosome negative myeloproliferative disorders. jms [Internet]. 2024Sep.27 [cited 2026Oct.2];27(3):10-7. Available from: https://www.jmsskims.org/index.php/jms/article/view/1396
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