Survival in hematological malignancies in the Nordic countries through a half century with correlation to treatment

Abstract

Studies of survival in hematological malignancies (HMs) have generally shown an improvement over time, although most of these studies are limited by a short follow-up period. Using the NORDCAN database with data from Denmark, Finland, Norway and Sweden, we follow periodic increases in relative survival in seven HMs through half a century up to 2015-2019. Five-year survival improved in all seven HMs, reaching 90% for Hodgkin lymphoma (HL), myeloproliferative neoplasias and chronic lymphocytic leukemia (CLL), 60% for multiple myeloma (MM) and chronic myeloid leukemias (CMLs), 50% for the myelodysplastic syndromes and 30% for acute myeloid leukemia (AML). Improvements in survival over 50 years ranged from 20% to more than 50% units across the different HMs. The likely reasons for such progress include earlier diagnoses, improved risk stratification and advances in treatment. We observed differing temporal trends in improvements in survival. The gradual increases observed in HL, CLL and AML highlight the impact of optimization of existing therapies and improvements in diagnostics and risk stratification, whereas the rapid increases observed in the CMLs and MM highlight the impact of novel therapies. Recent therapeutic advances may further improve survival in HMs where survival remains low such as in AML.

Fig. 1. Relative 5-year survival in hematological malignancies in the Nordic countries in 1970–1974 and 2015–2019 based on the NORDCAN database.

Note that the 1970–1974 datapoints were missing for MDS and for MPNs in DK and NO, and the symbols are marked as 0.

Fig. 2. Survival trends for Hodgkin lymphoma and multiple myeloma in the Nordic countries.

Relative 5-year survival in Hodgkin lymphoma (A) and multiple myeloma (B) in the Nordic countries from 1970 to 2019. The underlying data are available in Supplementary Table 1 with 95% CIs allowing assessment of significant improvements between subsequent periods. The introduction of novel therapies is shown on top of x-axis with details in Discussion. A MOPP nitrogen mustard, vincristine, procarbazide and prednisone, ABVD adriamycin, bleomycin, vinblastine, dacarbazine, BEAC (BEACOPP) bleomycin, etoposide, adriamycine, cyclophosphamide, vincristine, procarbazine, and prednisone, BREN Brentuximab, NIVO Nivolumab. B HSCT hematopoietic stem cell transplantation, HD-MELP high-dose melphalan, THAL thalodomine, BORT bortezomib, LENA lenalidomide, POMA pomalidomide, PANO panobinostat, CARF carfilzomib, DARA daratumumab.

Fig. 3. Survival trends for chronic lymphoid leukemia, myelodyplastic syndrome and myeloproliferative disease in the Nordic countries.

Relative 5-year survival in chronic lymphoid leukemia (A) and myelodysplastic syndrome and myeloproliferative disease (B) in the Nordic countries from 1970 to 2019. The underlying data are available in Supplementary Table 1 with 95% CIs allowing assessment of significant improvements between subsequent periods. The introduction of novel therapies is shown on top of x-axis with details in Discussion. A FLUD fludarabine, RITU rituximab, ALEM alemtuzumab, OFAT ofatumumab, BEND bendamustine, OBIN obinutuzumab, IDEL idelalisib, IBRU ibrutinib, VENA venetoclax.

Fig. 4. Survival trends for acute myeloid leukemia and chronic myeloid leukemia in the Nordic countries.

Relative 5-year survival in acute myeloid leukemia (A) and chronic myeloid leukemia (B) in the Nordic countries from 1970 to 2019. The underlying data are available in Supplementary Table 1 with 95% CIs allowing assessment of significant improvements between subsequent periods. The introduction of novel therapies is shown on top of x-axis with details in Discussion. A CYTA cytarabine+daunorubicin, HSCT hematopoietic stem cell transplantation, CPX CXP-351, GEMT gemtuzumab, MIDO midostaurin. B HYDR hydroxyurea, HSCT hematopoietic stem cell transplantation, IFN interferon alfa, IMAT imatinib mesylate, NILO nilotinib, DASA dasatinib.