[Analysis of clinical characteristics and prognosis of non-severe aplastic anemia children with chromosomal abnormalities].
Objective:To analyze the clinical characteristics and prognosis of non-severe aplastic anemia (NSAA) with chromosomal abnormalities in children.Method:A retrospective analysis of 304 cases with NSAA with successful karyotyping from 2001 to 2014 in the Institute of Hematology & Blood Disease Hospital was carried out. The treatment response, condition of blood transfusion were analyzed using χ2test, the cumulative survival was estimated by the Kaplan-Meier method.Result:Out of 304 patients, 28 patients had chromosomal abnormalities with trisomy 8 (7 cases, 25.0%), abnormalities in chromosome 7 (5 cases, 17.9%), and other types (16 cases, 57.1%). There were no significant differences in the treatment response(40.9% (9/22)vs. 58.6%(119/203), χ2=2.539,P=0.111), the rate of getting rid of blood transfusion(54.5%(6/11)vs. 65.0%(39/60), χ2=6.455,P=0.086), five-year progression-free survival (49.2%vs.70.8%, χ2=0.849,P=0.357), and five-year cumulative survival (79.1%vs. 92.8%, χ2=0.330,P=0.556) between the patients with or without chromosomal abnormalities. There were significant differences in the rate of disease progression(41.7%(10/24)vs. 22.3%(48/215), χ2=4.394,P=0.045), the incidence of myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) (20.8%(5/24)vs. 0.9%(2/215), χ2=30.082,P=0.000)and the five-year cumulative incidence of MDS or AML(33.4%vs. 0.8%, χ2=17.798,P=0.000)between children with and without chromosomal abnormalities.Conclusion:The incidence of chromosomal abnormalities in children with NSAA is 9.2%. The clinical features and treatment response are similar, but children with chromosomal abnormalities have a poorer prognosis, and have higher risk of progressing to MDS or AML.
Pediatric Blood Disease Center, Institute of Hematology & Blood Disease Hospital, Tianjin 300020, China.
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S
Zhu
W B
WB
An
Y
Y
Wan
Y Y
YY
Ren
R R
RR
Zhang
J L
JL
Zhang
C
C
Liu
C C
CC
Sun
L X
LX
Chang
X J
XJ
Chen
W Y
WY
Yang
Y
Y
Guo
Y M
YM
Chen
Y
Y
Zou
X F
XF
Zhu
D000293:Adolescent; D000741:Anemia, Aplastic / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D001803:Blood Transfusion; D002648:Child; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002898:Chromosomes, Human, Pair 8; D018450:Disease Progression; D018572:Disease-Free Survival; D006801:Humans; D007621:Karyotyping; D015470:Leukemia, Myeloid, Acute; D008297:Male; D009190:Myelodysplastic Syndromes; D011379:Prognosis; D012189:Retrospective Studies; D014314:Trisomy