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Methylthioadenosine phosphorylase deficiency in acute leukemia: pathologic,
cytogenetic, and clinical features
ST Traweek, MK Riscoe, AJ Ferro, RM Braziel, RE Magenis and JH Fitchen
Medical Research Service, Portland VA Medical Center, OR 97207.
Blast cells from 100 cases of acute leukemia were evaluated for the
presence of methylthioadenosine phosphorylase (MTAase), an enzyme important
in polyamine metabolism. Ten cases (10%) had undetectable levels of MTAase
activity. Of the 10, 5 had acute lymphoblastic leukemia (ALL), 3 had acute
myeloblastic leukemia (AML) and 2 expressed mixed lineage markers as
determined by immunophenotyping. A relatively high frequency (38%) of
MTAase deficiency was seen in ALL of T-cell origin. Nonmalignant
hematopoietic cells from three patients with MTAase-deficient leukemias had
readily detectable enzyme activity. Chromosomal abnormalities were detected
in four of the seven MTAase- deficient cases in which karyotypic analysis
was performed. No consistent karyotypic defect was apparent, and only one
case displayed changes in chromosome 9, the putative location of the MTAase
structural gene. The clinical findings among the enzyme-deficient cases
were unremarkable except that all patients were male (P less than .01).
Only one patient had "lymphomatous" features. We conclude that MTAase
deficiency occurs in a wide variety of acute leukemias, that the lack of
enzyme activity is specific to the malignant cells, and that an increased
incidence occurs in ALL of T-cell origin. Furthermore, no specific gross
chromosomal abnormality is associated with the enzyme deficiency. The
marked male predominance in patients with MTAase- deficient acute leukemias
suggests involvement of the X chromosome in the loss of enzyme activity.
The absence of MTAase in some leukemias may be therapeutically exploitable.
Volume 71,
Issue 6,
pp. 1568-1573,
06/01/1988
Copyright © 1988 by The American Society of Hematology
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