The characteristics and clinical outcome of adult patients with aplastic anemia and abnormal cytogenetics at diagnosis.
The characteristics and clinical outcome of 600 adult patients with aplastic anemia (AA) that had successful cytogenetic studies at the time of diagnosis were retrospectively evaluated. Among these, 572 (95.3%) had normal cytogenetics and 28 (4.7%) had abnormal cytogenetics. The most frequent abnormality was trisomy 8 (n = 15), followed by monosomy 7/deletion of 7q (n = 5), and deletion of 1q (n = 5). There were no statistically significant differences with respect to gender, hepatitis viral infection, paroxysmal nocturnal hemoglobinuria, or severity of disease between the patients in the normal and abnormal cytogenetics groups; however, the patients with abnormal cytogenetics were generally younger than those with normal cytogenetics (P < 0.001). Abnormal cytogenetics was associated with a higher cumulative leukemic transformation rate (P < 0.001) and lower leukemic transformation-free survival (P = 0.021). Furthermore, abnormal cytogenetics was an independent predictor of a poor response to immunosuppressive therapy (HR = 0.255; 95% CI = 0.077-0.839; P = 0.024). These analyses suggest that patients with AA and abnormal cytogenetics have different clinical characteristics compared to patients with AA and normal cytogenetics.
Department of Hematology/Oncology, KonKuk University Hospital, KonKuk University, Seoul, Korea.
Sung-Yong
SY
Kim
Jong-Wook
JW
Lee
Sung-Eun
SE
Lee
Byung-Sik
BS
Cho
Myungshin
M
Kim
Ki-Seong
KS
Eom
Yoo-Jin
YJ
Kim
Hee-Je
HJ
Kim
Seok
S
Lee
Chang-Ki
CK
Min
Seok-Goo
SG
Cho
Dong-Wook
DW
Kim
Kyungja
K
Han
Woo-Sung
WS
Min
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000741:Anemia, Aplastic / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002471:Cell Transformation, Neoplastic; D002869:Chromosome Aberrations; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014314:Trisomy; D055815:Young Adult
D007166:Immunosuppressive Agents