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How unique is pure erythroid leukaemia? A retrospective analysis of seven cases and review of the literature
  1. Eric Wong1,
  2. Victoria Ling2,
  3. David Westerman3,4,
  4. Susan Morgan3,
  5. Surender Juneja1,4
  1. 1Department of Diagnostic Haematology, Royal Melbourne Hospital, Melbourne, Victoria, Australia
  2. 2Department of Diagnostic Haematology, Alfred Hospital, Melbourne, Victoria, Australia
  3. 3Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia
  4. 4University of Melbourne, Melbourne, Victoria, Australia
  1. Corresponding to Dr Eric Wong, Department of Diagnostic Haematology, Royal Melbourne Hospital, Parkville, VIC 3050, Australia; eric.wong{at}mh.org.au

Abstract

Aims Pure erythroid leukaemia (PEL) is a rare subtype of acute myeloid leukaemia (AML) and its clinicopathological features are not well-defined. The aim of this study was to describe the immunophenotypic, cytogenetic and clinical features of PEL and to compare these with cases of AML with ≥50% erythroblasts.

Methods Cases of PEL according to WHO morphological criteria diagnosed at three institutions from 1997 to 2013 were included. A comparison cohort comprised of AML with ≥50% erythroblasts. The clinical, histopathology, immunophenotypic and cytogenetic features of cases were analysed. We also reviewed the existing literature on PEL, and combined our cohort with previously reported cases of PEL in a pooled analysis.

Results There were seven cases of PEL diagnosed at our institutions. There was a high incidence of either prior chemoradiotherapy exposure or evolution from pre-existing myelodysplastic syndrome (MDS) (71%). The leukaemic blasts frequently expressed glycophorin C (100%), CD117 (83%) and were myeloperoxidase negative (83%). Complex karyotypes were present in 83% of cases. Median overall survival was 2.9 months. Compared with AML with ≥50% erythroblasts, cases of PEL demonstrated a higher incidence of adverse-risk cytogenetics (p=0.01) and prior exposure to chemoradiotherapy (p=0.01).

Conclusions PEL appears to be a unique entity that is often secondary or therapy related, commonly features a complex karyotype and has a poor prognosis. It is morphologically and immunophenotypically distinct from other cases of AML with erythroid hyperplasia.

  • LEUKAEMIA
  • HISTOPATHOLOGY
  • IMMUNOPHENOTYPING
  • CYTOGENETICS

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