Prognostic scoring systems and comorbidities in chronic myelomonocytic leukaemia: a nationwide population‐based study

Abstract

Summary Outcomes in chronic myelomonocytic leukaemia (CMML) are highly variable and may be affected by comorbidity. Therefore, prognostic models and comorbidity indices are important tools to estimate survival and to guide clinicians in individualising treatment. In this nationwide population‐based study, we assess comorbidities and for the first time validate comorbidity indices in CMML. We also compare the prognostic power of: the revised International Prognostic Scoring System (IPSS‐R), CMML‐specific prognostic scoring system (CPSS), MD Anderson Prognostic Scoring System (MDAPS) and Mayo score. In this cohort of 337 patients with CMML, diagnosed between 2009 and 2015, the median overall survival was 21·3 months. Autoimmune conditions were present in 25% of the patients, with polymyalgia rheumatica and Hashimoto’s thyroiditis being most common. Of the tested comorbidity indices: the Charlson Comorbidity Index (CCI), Haematopoietic cell transplantation‐specific Comorbidity Index (HCT‐CI) and Myelodysplastic Syndrome‐Specific Comorbidity Index (MDS‐CI), CCI had the highest C‐index (0·62) and was the only comorbidity index independently associated with survival in multivariable analyses. When comparing the prognostic power of the scoring systems, the CPSS had the highest C‐index (0·69). In conclusion, using ‘real‐world’ data we found that the CCI and CPSS have the best prognostic power and that autoimmune conditions are overrepresented in CMML.

Impact of Mutational Status and Prognostic Factors on Survival in Chronic Myelomonocytic Leukemia With Systemic Inflammation and Autoimmune Disorders

“…Regarding prognosis, in the whole cohort, SIAD were not associated with a pejorative prognosis, in accordance with other studies 10,13 . We showed that among CMML patients with SIAD, autoimmune cytopenia had a significantly better OS compared with other types of SIAD.…”

“…CPSS and CPSS‐Mol scores, considered as the most relevant scoring systems for CMML in a contemporary practice, were not applicable in SIAD patients 13 . This result suggests that even though the mutational landscape was similar in CMML patients with or without SIAD, other factors have an impact on these scores.…”

“…Regarding prognosis, in the whole cohort, SIAD were not associated with a pejorative prognosis, in accordance with other studies. 10,13 We showed that among CMML patients with SIAD, autoimmune cytopenia had a significantly better OS compared with other types of SIAD. The similar mutational landscape between each SIAD subtype suggests that this difference may not be explained by mutations studied in our work.…”

Autoimmune Complications in Hematologic Neoplasms

“…Systemic AIDs are described in up to 25% of CMML patients, again mainly systemic vasculitis, followed by connective tissue diseases [57,68,76]. The majority of cases are CMML-1, with a progression to acute leukemia comparable with that of patients without AID [57].…”

Income, education and their impact on treatments and survival in patients with myelodysplastic syndromes

“…From 2012 and onwards, follow‐up data at 1 year and every third year after diagnosis is recorded. Compared to the Swedish Cancer Register, to which reporting is mandatory for clinicians and pathologists, the coverage was 97% for the years 2009‐2015 16 . In our data, we had access to 1‐year follow‐up for 1562 patients (53%), 3‐year follow‐up for 523 patients (18%) and 6‐year follow‐up for 26 patients (1%).…”