Atypical chronic myeloid leukaemia: A case of an orphan disease—A multicenter report by the Polish Adult Leukemia Group

• Published in: Hematological oncology Vol. 36; no. 3; pp. 570 - 575
• Main Authors: Drozd‐Sokołowska, Joanna, Mądry, Krzysztof, Waszczuk‐Gajda, Anna, Biecek, Przemysław, Szwedyk, Paweł, Budziszewska, Katarzyna, Raźny, Magdalena, Dutka, Magdalena, Obara, Agata, Wasilewska, Ewa, Lewandowski, Krzysztof, Piekarska, Agnieszka, Bober, Grażyna, Krzemień, Helena, Stella‐Hołowiecka, Beata, Kapelko‐Słowik, Katarzyna, Sawicki, Waldemar, Paszkowska‐Kowalewska, Małgorzata, Machowicz, Rafał, Dwilewicz‐Trojaczek, Jadwiga
• Format: Journal Article
• Language: English
• Published: England Wiley Subscription Services, Inc 01.08.2018
• Subjects: Acute myeloid leukemia; atypical chronic myeloid leukaemia; Chronic myeloid leukemia; Diagnostic systems; Hematopoietic stem cells; Hemoglobin; Leukemia; Leukocytes; MDS/MPN‐U; Medical prognosis; myelodysplastic/myeloproliferative neoplasms; Neoplasms; Patients; Stem cell transplantation; Stem cells; Survival; Transplantation; Transplants & implants; MDS/MPN-U; myelodysplastic/myeloproliferative neoplasms; atypical chronic myeloid leukaemia
• DOI: 10.1002/hon.2501
• ISSN: 0278-0232

Atypical 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.