Journal of Clinical Oncology, Vol 1, 669-676, Copyright © 1983 by American Society of Clinical Oncology
Philadelphia chromosome and terminal transferase-positive acute leukemia: similarity of terminal phase of chronic myelogenous leukemia and de novo acute presentation
K Jain, Z Arlin, R Mertelsmann, T Gee, S Kempin, B Koziner, A Middleton, S Jhanwar, R Chaganti and B Clarkson
Twenty-eight patients 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 months in the responders. Clinical presentation,
laboratory data, cytogenetics, response to treatment, and survivals of the
two groups of patients are compared. These results appear to be similar,
suggesting a common or closely related origin. Since the overall survival
of those receiving chemotherapy maintenance is poor, three 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 + months) 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.
