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Related Experiment Videos

Typical essential thrombocythaemia does not express bcr-abelson fusion transcript.

G Damaj1, E Delabesse, C Le Bihan

  • 1Service d'Hématologie, Service d'Hématologie Biologique, Département de Biostatistique et d'Informatique Médicale, and Laboratoire de Cytogénétique, Hôpital Necker Enfants-Malades, Paris, France. damajg@marseille.fnclcc.fr

British Journal of Haematology
|March 12, 2002
PubMed
Summary
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Essential thrombocythaemia (ET) rarely shows the bcr-abl transcript. However, some ET patients with basophilia may represent a new variant, potentially bridging chronic myeloid leukaemia and typical ET.

Area of Science:

  • Hematology
  • Oncology
  • Molecular Biology

Background:

  • Essential thrombocythaemia (ET) is a chronic myeloproliferative disorder (MPD) defined by high platelet counts without a clear cause.
  • Diagnostic criteria exclude features of other MPDs, such as the Philadelphia chromosome (Ph) or bcr-abl rearrangement.
  • Recent reports of bcr-abl transcript positivity in ET are controversial.

Observation:

  • This study investigated bcr-abl transcript presence in 121 ET patients.
  • The bcr-abl transcript was detected in only two patients, one with Ph at diagnosis.
  • Mild basophilia was noted in both bcr-abl-positive cases.

Findings:

  • The bcr-abl transcript is rare in well-defined ET.
  • A subset of ET patients with basophilia may express the bcr-abl transcript.

Related Experiment Videos

  • Two bcr-abl-negative patients developed Ph-negative acute myeloid leukaemia (AML); one bcr-abl-positive patient died from AML.

    • Implications:

    • bcr-abl-expressing ET with basophilia might represent a distinct entity.
    • This entity could be intermediate between chronic myeloid leukaemia (CML) and typical ET.
    • Further prospective studies are needed to characterize bcr-abl-expressing ET.