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  • Title: Lymphoid blast crisis in a patient with Philadelphia-chromosome-negative chronic myelocytic leukemia.
    Author: Takahira H, Shibata K, Hirata J, Umemura T, Nishimura J, Nawata H.
    Journal: Acta Haematol; 1989; 81(3):155-9. PubMed ID: 2496564.
    Abstract:
    A 24-year-old man with Philadelphia-chromosome (Ph)-negative chronic myelocytic leukemia (CML) developed lymphoid blast crisis. In the chronic phase, karyotype was normal and the clinical and hematological features were indistinguishable from those of Ph-positive CML. Rearrangement of the breakpoint cluster region (bcr) was observed. In the blast phase, blast cells showed early B-cell phenotype (CALLA+, Ia+, TdT+) with a rearranged immunoglobulin heavy-chain gene joining region (JH). By using an immunoblotting method and antiphosphotyrosine sera, P210bcr-abl protein was detected. The patient responded well to vincristine and prednisolone (VP) therapy. These findings support the concept that Ph-negative bcr+ CML can behave in a very similar fashion to Ph-positive CML, not only in the clinical features of the chronic phase but also in the manner of the blast crisis.
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