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Ph-negative chronic myeloid leukaemia.

P H Fitzgerald, M E Beard, C M Morris

    British Journal of Haematology
    |July 1, 1987
    PubMed
    Summary
    This summary is machine-generated.

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    Philadelphia chromosome-negative chronic myeloid leukemia (CML) patients may present similarly to Ph-positive CML. Some cases require reclassification as chronic myelomonocytic leukemia (CMML), while others show molecular defects mirroring Ph-positive CML.

    Area of Science:

    • Hematology
    • Molecular Biology
    • Oncology

    Background:

    • Chronic myeloid leukemia (CML) is often characterized by the Philadelphia chromosome (Ph).
    • Distinguishing Ph-negative CML from other myeloproliferative neoplasms can be challenging.
    • Understanding molecular alterations is crucial for accurate diagnosis and prognosis.

    Purpose of the Study:

    • To analyze the clinical, hematological, and molecular characteristics of patients with Philadelphia chromosome-negative chronic myeloid leukemia (CML).
    • To determine if molecular defects associated with Ph-positive CML can occur in Ph-negative CML patients.
    • To assess the clinical course and prognosis of Ph-negative CML patients with specific molecular findings.

    Main Methods:

    • Case series analysis of five patients with Ph-negative CML.

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  • Clinical and hematological assessments.
  • Molecular analysis to detect gene rearrangements, specifically the c-abl and bcr gene juxtaposition.

    • Main Results:

    • Two of five patients were clinically and hematologically indistinguishable from Ph-positive CML.
    • Three patients were reclassified as chronic myelomonocytic leukemia (CMML).
    • The two clinically similar patients exhibited the c-abl/bcr gene fusion characteristic of Ph-positive CML; the other three did not.

    Conclusions:

    • Ph-negative CML can present with clinical and hematological features similar to Ph-positive CML.
    • Some rare patients with Ph-negative CML may harbor the molecular defect (c-abl/bcr gene fusion) typically seen in Ph-positive CML.
    • The clinical course and prognosis for patients with the Ph molecular defect but without the Philadelphia chromosome appear similar to those with Ph-positive CML.