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

Abstract

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.
Author Joanna Drozd-Sokołowska
Joanna Drozd-Sokołowska,,
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, Krzysztof Mądry
Krzysztof Mądry,,
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, Anna Waszczuk-Gajda
Anna Waszczuk-Gajda,,
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, Przemysław Biecek (FMIS / DCSDCAM)
Przemysław Biecek,,
- Department of CAD/CAM Systems Design and Computer-Aided Medicine
, Paweł Szwedyk
Paweł Szwedyk,,
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, Katarzyna Budziszewska
Katarzyna Budziszewska,,
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, Magdalena Raźny
Magdalena Raźny,,
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, Magdalena Dutka
Magdalena Dutka,,
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, Agata Obara
Agata Obara,,
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, Ewa Wasilewska
Ewa Wasilewska,,
-
et al.`
Journal seriesHematological Oncology, ISSN 0278-0232, (A 25 pkt)
Issue year2018
Vol36
No3
Pages570-575
Publication size in sheets0.5
ASJC Classification1306 Cancer Research; 2730 Oncology; 2720 Hematology; 2700 General Medicine
DOIDOI:10.1002/hon.2501
URL https://onlinelibrary.wiley.com/doi/epdf/10.1002/hon.2501
Languageen angielski
Score (nominal)25
ScoreMinisterial score = 25.0, 23-09-2019, ArticleFromJournal
Publication indicators Scopus SNIP (Source Normalised Impact per Paper): 2016 = 0.466; WoS Impact Factor: 2017 = 3.193 (2) - 2017=2.423 (5)
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