title: Childhood acute promyelocytic leukemia: a rare variant of nonlymphoid leukemia with distinctive clinical and biologic features.
abstract: Of 251 consecutive cases of childhood acute nonlymphocytic leukemia (ANLL) seen at St. Jude Children's Research Hospital over a 12-year period, 16 (6.4%) were classified as promyelocytic according to the French-American-British definition. Patients with this form of leukemia were older at diagnosis than the group representing all other ANLL subtypes (median age, 14.8 vs. 9.0 years); they had lower leukocyte counts (median, 4.5 vs. 25.9 x 10(9)/liter), and a higher percentage were girls (68% vs. 44%). They also were much more likely to have a coagulation abnormality (75% vs. 13%). Only 44% of the promyelocytic group achieved complete remission, compared with 79% of the remaining patients (p = 0.001); however, after a median follow-up of 3.5 years, all but two of the responding patients with promyelocytic leukemia remain in complete remission. The majority of induction failures in the promyelocytic group (six of nine) resulted from complications that developed during periods of marrow hypoplasia or before hypoplasia was induced; whereas in the comparison group, more than half of the patients who failed had evidence of absolute or relative drug resistance. It is concluded that acute promyelocytic leukemia in children differs sufficiently from other subtypes of childhood ANLL to justify clinical trials of selective therapy. Recommendations for the use of heparin and blood component support in these patients are given.
authors.affiliation: Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101.
authors.forename: M
authors.initials: M
authors.lastname: Carter
authors.forename: D K
authors.initials: DK
authors.lastname: Kalwinsky
authors.forename: G V
authors.initials: GV
authors.lastname: Dahl
authors.forename: V M
authors.initials: VM
authors.lastname: Santana
authors.forename: C A
authors.initials: CA
authors.lastname: Mason
authors.forename: M J
authors.initials: MJ
authors.lastname: Schell
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy; D005260:Female; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D015473:Leukemia, Promyelocytic, Acute / Q000145:classification / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D011379:Prognosis
chemical_list: D006493:Heparin