Page 147 - Haematologica3

Introduction
Over recent decades, new treatment options have emerged in myeloma, with great expectations of improved survival. The introduction of high-dose melpha- lan with autologous stem cell support (HDM-ASCT) and newer drugs, such as the immunomodulatory agents (thalidomide, lenalidomide, and pomalidomide), protea- some inhibitors (bortezomib and carfilzomib), monoclon- al antibodies, and other classes, has led to a rapid imple- mentation of these drugs under international guidelines.1-7 To date, most studies on myeloma are based on selected patients from large referral centers and collaborative groups, with defined inclusion and exclusion criteria. But these often omit elderly patients, and thus do not reflect the true 'real-world' population.8 Also, there is limited information available on the use of new therapies and their efficacy and tolerability in standard practice, sup- porting the need for representative population-based prospective studies on characteristics, diagnostics, treat- ment and outcome in myeloma patients.
Survival data from cancer registries are available, but often lack information on baseline characteristics and treatment. EUROCARE, covering nearly 50% of patients diagnosed with plasma cell neoplasms in Europe in the period 2000-2007, reports an age-standardized 5-year rel- ative survival (RS) of 39.2%, an increase from 29.8% in 1997. Outcome was significantly better in the younger patients (68.6% vs. 21.8% 5-year relative survival), and in women (40.4% vs. 38.1%).9 These results have later been confirmed by other cancer registry data.10-12
A 2010 Swedish study of retrospective data regarding baseline characteristics and treatment of consecutive patients in Malmö found a similar trend in improved sur- vival, which correlated with the introduction of new treat- ment modalities.13,14
The Swedish Myeloma Registry was established in 2008, and the first Swedish guidelines on diagnostics and treatment of myeloma were published in 2010. This is the first report on our population-based data on characteris- tics, treatment and survival in Swedish myeloma patients diagnosed from January 2008 through December 2015.
Methods
The Swedish Cancer Registry
The Swedish Cancer Registry is a nation-wide compulsory dual-report system developed in 1958, which is supported by the personal identification code system used for all Swedish citizens which was established in 1947. First, all pathology specimens indi- cating malignancy are reported by the pathologist to the Regional Tumor Registry. Second, data on date and type of cancer diagnosis of all patients with a newly diagnosed cancer are reported by cli- nicians, with missing data actively requested to secure a high level of completeness. In a validation study, the completeness (95%) and diagnostic accuracy (98%) of the Swedish Cancer Registry was found to be very high for multiple myeloma patients.15
The Swedish Myeloma Registry
The Swedish Cancer Registry comprises web-reported clinical and laboratory data on all patients diagnosed with active myelo- ma, smoldering myeloma, plasma cell leukemia, and solitary bone and extramedullary plasmocytomas in Sweden since 2008, at time of diagnosis and after a 1-year follow up. Coverage is analyzed
Table 1. Characteristics of active myeloma (MM) and smoldering myeloma (SMM) patients in the Swedish Myeloma Registry.
Experiences from the Swedish Myeloma Registry
Characteristics
Total, n (%) Diagnosis, n (%)
Multiple myeloma
Smoldering multiple myeloma Age in years at dx, median
All Male Female
Immunoglobuline class n (%) IgG
IgA
Bence-Jones MM Non-secretory MM IgD
IgM
Not known
More than one Ig IgE
n: number; dx: diagnosis.
Patients
4904 (100%)
3988 (81.3%) 916 (18.6%)
71 71 73
2882 (58.8) 1033 (22.3) 688 (14.0) 143 (2.9) 19 (0.4) 14 (0.3) 23 (0.5) 41 (0.8)
1 (0.0)
haematologica | 2018; 103(3)
through the compulsory Swedish Cancer Registry. Survival data are obtained from the Swedish Population Registry. Patients diag- nosed by autopsy are included in the Swedish Cancer Registry, but not in the Swedish Myeloma Registry. The registry is publicly financed, and the patients are reported by treating physicians and nurses. Courses are held for those responsible for reporting patient data to assure coherent reporting in all regions and hospitals. These courses cover inclusion criteria, parameters, and the manual of the Swedish Myeloma Registry. Criteria for the diagnosis of active myeloma (MM), smoldering myeloma (SMM), plasmocy- toma, and plasma cell leukemia are defined according to the International Myeloma Working Group (2003).16 Other gam- mopathies, such as monoclonal gammopathy of undetermined significance (MGUS) and AL-amyloidosis are not included in the registry. Age-specific incidence, age distribution at diagnosis, median time from diagnosis to registry report, and distribution of the diagnoses in the registry are reported. Adherence to treatment guidelines concerning diagnostics and ISS-staging (International Staging System) is checked by studying the use of different diag- nostic tools such as bone marrow sample, cytogenetics including fluorescence in situ hybridization (FISH), β 2-microglobulin (β2m) and s-albumin. Baseline characteristics at diagnosis are collected, including M-protein isotype, percentage of plasma cells in the bone marrow, serum free-light chain (FLC), and laboratory param- eters capturing CRAB criteria (CRAB; Calcium, Renal insufficien- cy, Anemia or Bone lesions). One year after diagnosis of sympto- matic MM, data on first-line therapy, occurrence and date of first relapse or complications are requested. The study was performed in agreement with the ethics committee of Stockholm and the Swedish Society of Hematology.
Treatment of MM in Sweden
In Sweden, patients with myeloma are typically diagnosed and followed clinically by physicians at hospital-based hematology centers, and no patients are seen at private hospitals. In the study period, the treatment of MM was guided by the British/Nordic
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