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Blood, Vol. 95 No. 1 (January 1), 2000:
pp. 90-95
Clinical description of 44 patients with acute promyelocytic
leukemia who developed the retinoic acid syndrome
Martin S. Tallman,
Janet W. Andersen,
Charles A. Schiffer,
Frederick R. Appelbaum,
James H. Feusner,
Angela Ogden,
Lois Shepherd,
Jacob
M. Rowe,
Christopher François,
Richard S. Larson, and
Peter H. Wiernik
From Eastern Cooperative Oncology Group, Northwestern University
Medical School, Robert H. Lurie Comprehensive Cancer Center, Chicago
IL; Eastern Cooperative Oncology Group, Harvard School of Public
Health, Division of Biostatistics, Boston MA; Cancer & Leukemia Group
B, Wayne State University, Barbara Ann Karmanos Cancer Center, Detroit,
MI; Southwest Oncology Group, University of Washington, Fred Hutchinson
Cancer Research Center, Seattle WA; Children's Cancer Group,
Children's Hospital Oakland, Oakland, CA; Pediatric Oncology Group,
Texas Children's Hospital, Houston TX; National Cancer Institute of
Canada Clinical Trials Group, Kingston, Ontario; Eastern Cooperative
Oncology Group, University of Rochester, Rambam Medical Center, Haifa,
Israel; Southwestern Oncology Group, University of New Mexico,
Department of Pathology, Albuquerque, NM; Eastern
Cooperative Oncology Group, Our Lady of Mercy Cancer Center, New York
Medical College, Bronx NY.
We examined the incidence, clinical course, and outcome of patients
with newly diagnosed acute promyelocytic leukemia (APL) who developed
the retinoic acid syndrome (RAS) treated on the Intergroup
Protocol 0129, which prospectively evaluated the role of
alltrans retinoic acid (ATRA) alone during induction and as maintenance therapy. Forty-four of 167 (26%) patients receiving ATRA
for induction developed the syndrome at a median of 11 days of
ATRA (range, 2-47). The median white blood cell (WBC) count was
1450/µL at diagnosis and was 31 000/µL (range, 6800-72 000/µL) at the time the syndrome developed. ATRA was discontinued in 36 of the
44 patients (82%) and continued in 8 patients (18%), with subsequent
resolution of the syndrome in 7 of the 8. ATRA was resumed in 19 of the 36 patients (53%) in whom ATRA was stopped and not in 17 (47%). The syndrome recurred in 3 of those 19 patients, with 1 death
attributable to resumption of the drug. Ten of these 36 patients
received chemotherapy without further ATRA, and 8 achieved complete
remission (CR). Among 7 patients in whom ATRA was not restarted and
were not treated with chemotherapy, 5 achieved CR and 2 died. Two
deaths were definitely attributable to the syndrome. No patient
receiving ATRA as maintenance developed the syndrome. (Blood.
2000;95:90-95)
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