Philadelphia chromosome and terminal transferase-positive acute leukemia: similarity of terminal phase of chronic myelogenous leukemia and de novo acute presentation.

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Abstract
Patients (28) with Philadelphia chromosome (Ph1)-positive and terminal transferase (TdT)-positive acute leukemia (AL) were treated with intensive chemotherapy used for adult acute lymphoblastic leukemia (L-10 and L-10M protocols). Fifteen patients had a documented chronic phase of Ph1-positive chronic myelogenous leukemia preceding the acute transformation (TdT + BLCML) while the remaining 13 patients did not (TdT + Ph1 + AL). An overall complete remission (CR) rate of 71% was obtained with a median survival of 13 mo. in the responders. Clinical presentation, laboratory data, cytogenetics, response to treatment and survivals of the 2 groups of patients are compared. The results appear to be similar, suggesting a common or closely related origin. Since the overall survival of those receiving chemotherapy maintenance is poor, 3 patients underwent allogeneic bone marrow transplantation (BMT) from histocompatibility leukocyte antigen-matched siblings after they achieved CR. One of them is a long-term survivor (35 + mo.) with a Ph1-negative bone marrow. New techniques such as BMT should be considered in young patients with a histocompatibility leukocyte antigen-compatible sibling once a CR has been achieved.