Acute leukaemia with a pure erythroid phenotype: Under-recognized morphological and cytogenetic signatures associated universally with primary refractory disease and a dismal clinical outcome Journal Article
| Authors: | Park, D. C.; Ozkaya, N.; Lovitch, S. B. |
| Article Title: | Acute leukaemia with a pure erythroid phenotype: Under-recognized morphological and cytogenetic signatures associated universally with primary refractory disease and a dismal clinical outcome |
| Abstract: | Aims: Pure erythroid leukaemia (PEL) is an extremely rare and aggressive subtype of acute myeloid leukaemia defined by the World Health Organization (WHO) as a neoplastic proliferation of immature cells committed exclusively to the erythroid lineage, comprising >80% of bone marrow cells and not meeting the criteria of other well-defined myeloid neoplasms. The aim of this study was to describe the clinicopathological features of acute leukaemias with a pure erythroid phenotype (ALPEP) irrespective of their WHO classification and to determine if ALPEP represents a distinct clinicopathological entity. Methods and results: We identified seven cases of ALPEP, in which immature cells fulfilled WHO morphological and immunophenotypical criteria for PEL. All patients except one were male, with a median age of 60 years. Three cases represented de novo PEL, three were therapy-related myeloid neoplasms and one was a blast phase of a myeloproliferative neoplasm. Extensive tumour necrosis was present in five cases (71%). Five cases with available modal karyotypes all demonstrated a complex karyotype involving the TP53 gene locus, with three cases (60%) also showing a monosomy 5 or deletion 5q and additional material on chromosome 19q13. All patients died of their disease, with a mean overall survival of 189 and 64.7 days in cases without and with necrosis on the initial biopsy, respectively. Conclusions: We describe previously unreported but relatively common findings of extensive tumour necrosis and recurring cytogenetic abnormalities in ALPEP. Our findings suggest strongly that ALPEP represents a distinct clinicopathological entity regardless of its WHO classification. © 2017 John Wiley & Sons Ltd |
| Keywords: | tp53 gene; bone marrow necrosis; complex karyotype; m6b; pure erythroid leukemia |
| Journal Title: | Histopathology |
| Volume: | 71 |
| Issue: | 2 |
| ISSN: | 0309-0167 |
| Publisher: | Wiley Blackwell |
| Date Published: | 2017-08-01 |
| Start Page: | 316 |
| End Page: | 321 |
| Language: | English |
| DOI: | 10.1111/his.13207 |
| PROVIDER: | scopus |
| PUBMED: | 28261852 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 1 August 2017 -- Source: Scopus |
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