Demographics and survival in atypical chronic myeloid leukemia: SEER data analysis.
Clinical Lymphoma, Myeloma & Leukemia
Context: Atypical chronic myeloid leukemia (aCML) or BCR/ ABL 1 negative CML is an uncommon myelodysplastic syndrome (MDS)/ myeloproliferative neoplasm (MPN). Due to the rarity of this disease, information on the natural history is limited and there are no current standards of care available.Objective: To determine impact of demographics and treatment on overall and disease-specific survival of aCML patients. Design: This was a retrospective analysis of all aCML cases diagnosed between 2005 and 2015 in the SEER database, which contains incidence and population data. Patients were identified using ICD histology codes. JMP14 was used to generate Kaplan-Meier curves and a proportional hazards model for survival analysis. Main outcome measures: Overall, disease-specific survival and comparative survival of treated and untreated aCML patients. Results: Over eleven years (2005-2015), 151 patients were diagnosed with aCML. Most were males (92, 61%) and Caucasian (117, 77.5%). Median age was 71 years (IQR 61-80). The median is reported as the SEER database does not record specific ages for 85 years and above. Eighteen patients were aged 85 and above in the current sample (12%). During the study period, overall mortality was 77% (116) of which 63% (74) were attributable to aCML. Five-year overall survival was 14%, with a median survival of 14 months; aCML-specific survival was 28% at 5 years, with a median survival of 24 months. Of the 151 cases, 78% received chemotherapy while 22% did not. Comparative survival of patients receiving chemotherapy versus untreated patients showed improved survival within the first year, but no difference thereafter (log-rank p¼0.68). Adjusted analysis for age and sex showed a non-significant 10% increase in survival with chemotherapy (HR 0.9, 95%CI 0.58-1.44, p¼0.66). The only significant predictor of survival was age (HR 1.04 per unit increase, 95%CI 1.02-1.05, p<0.0001), whereas male sex was associated with a non-significant increase in mortality (HR 1.03, 95%CI 0.7-1.5, p¼0.9). Conclusion: a CML is a rare disease, more common in the elderly, in males and in the Caucasian population. Survival is poor and it does not appear to be impacted by treatment.
Anusim N, Ionescu F, Ezekwudo D, Jaiyesimi I. Demographics and Survival in Atypical Chronic Myeloid Leukemia: SEER Data Analysis. Clinical Lymphoma, Myeloma and Leukemia. 2019 Sep 1;19:S345-6.