Atypical chronic myeloid leukaemia: A case of an orphan disease-A multicenter report by the Polish Adult Leukemia Group
Joanna Drozd-Sokołowska , Krzysztof Mądry , Anna Waszczuk-Gajda , Przemysław Biecek , Paweł Szwedyk , Katarzyna Budziszewska , Magdalena Raźny , Magdalena Dutka , Agata Obara , Ewa Wasilewska , Krzysztof Lewandowski , Agnieszka Piekarska , Grażyna Bober , Helena Krzemień , Beata Stella-Hołowiecka , Katarzyna Kapelko-Słowik , W. Sawicki , Małgorzata Paszkowska-Kowalewska , Rafał Machowicz , Jadwiga Dwilewicz-Trojaczek
AbstractAtypical chronic myeloid leukaemia (aCML) belongs to myelodysplastic/myeloproliferative neoplasms. Because of its rarity and changing diagnostic criteria throughout subsequent classifications, data on aCML are very scarce. Therefore, we at the Polish Adult Leukemia Group performed a nationwide survey on aCML. Eleven biggest Polish centres participated in the study. Altogether, 45 patients were reported, among whom only 18 patients (40%) fulfilled diagnostic criteria. Among misdiagnosed patients, myelodysplastic/myeloproliferative syndrome unclassifiable and chronic myelomonocytic leukaemia were the most frequent diagnoses. Thirteen patients were male, median age 64.6 years (range 40.4‐80.9). The median parameters at diagnosis were as follows: white blood cell count 97 × 109/L (23.8‐342) with immature progenitors amounting at 27.5% (12‐72), haemoglobin 8.6 g/dL (3.9‐14.9), and platelet count 66 × 109/L (34‐833). Cytoreductive treatment was used in all patients, and 2 patients underwent allogeneic hematopoietic stem cell transplantation. The median overall survival was 14.1 months (95% CI, 7.2), with median acute myeloid leukaemia‐free survival of 13.3 months (95% CI, 3.6‐22.6). Cumulative incidence of acute myeloid leukaemia transformation after 1 year in aCML group was 12.5% (95% CI, 0%‐29.6%). To conclude, aCML harbours a poor prognosis. Treatment options are limited, with allogeneic hematopoietic stem cell transplantation being the only curative method at present, although only a minority of patients are transplant eligible. Educational measures are needed to improve the quality of diagnoses.
|Journal series||Hematological Oncology, ISSN 0278-0232|
|Publication size in sheets||0.5|
|ASJC Classification||; ; ;|
|Score||= 25.0, 16-06-2020, ArticleFromJournal|
|Publication indicators||= 1; = 3; : 2018 = 0.621; : 2018 = 3.439 (2) - 2018=2.658 (5)|
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