Acute Erythroid leukaemias

2002-07-01   Estella Matutes , Sally B Killick 

1.Department of Haematology, St George s Hospital Medical School, London, UK
2.Department of Haematology, St Georges Hospital Medical School, London, UK

Clinics and Pathology

Epidemiology

Acute erythroid leukaemia is an uncommon form of acute myeloid leukaemia (AML), accounting for approximately 3-4% of cases. Perhaps more than other subtypes of AML, it may represent the evolution or transformation of a myelodysplastic syndrome (MDS), and may be secondary to previous chemotherapy, immunosuppressive treatment or radiotherapy given for a wide range of malignant or non-malignant diseases. It is more commonly associated with exposure to alkylating agents or benzene than other subtypes of AML.

Clinics

Acute erythroid leukaemia presents with symptoms and signs of cytopenias. It is more common in adults than in children.

Cytology

Immunophenotype
  • Erythroleukaemia: The myeloid blasts express a variety of myeloid markers, similar to othe subtypes of AML ­ CD13, CD33, CD117 (ckit) and MPO. The erythroblasts lack myeloid antigens but are positive to glycophorin A.
  • Pure erythroid leukaemia: Erythroid blasts which have differentiated will be positive with glycophorin A but negative with MPO and myeloid markers. The more immature blasts are difficult to identify as erythroid because they are usually negative for glycophorin A. Immature erythroid progenitors may be detected using carbonic anhydrase 1 or CD36. Although CD36 is not specific for erythroid progenitors, negative markers for megakaryocytes and monocytes will aid the diagnosis.
  • Treatment

    The prognosis of acute erythroid leukaemia is reported as poor. It is, however, important to differentiate de novo from secondary or therapy related erythroid leukaemia, where the later have a worse prognosis. Remission induction for de novo disease is similar to other subtypes of AML, however the poor outcome has been linked to short remission duration. Patients with complex karyotypes or abnormalities of chromosomes 5 and/or 7 have a higher relapse rate than those with normal or simple karyotypes. Data from the Medical Research Council AML10 trial show reduced relapse rates in patients with both standard and poor risk AML after autologous bone marrow transplantation. It may, therefore, be reasonable to consider early stem cell transplantation in first complete remission in patients with acute erythroid leukaemia, particularly those with a poor risk karyotype.

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    Summary

    Note

    Criteria for diagnosis of acute erythroid leukaemia

    Citation

    Estella Matutes ; Sally B Killick

    Acute Erythroid leukaemias

    Atlas Genet Cytogenet Oncol Haematol. 2002-07-01

    Online version: http://atlasgeneticsoncology.org/haematological/1215/m6anllid1215

    External Links