Clinical data on 29 Down syndrome patients with refractory/relapsed myeloid leukemia
No. . | Clinical characteristics at initial diagnosis of ML-DS . | GATA1 status . | IF/RL . | Time to IF/RL, mo* . | Subsequent CR . | HSCT . | Survival, mo† . | Cause of death . | ||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
Sex . | Age, y . | WBC, /μL . | FAB . | Karyotype . | ||||||||
1 | F | 3 | 3000 | M7 | Constitutional | NE | RL | 4 | No | No | 5 | Leukemia |
2 | M | 3 | 51 700 | M7 | Constitutional | NE | IF | 1 | No | UCBT | 8 | Leukemia |
3 | M | 2 | 10 000 | M7 | Other | NE | RL | 2 | No | No | 3 | Leukemia |
4 | F | 2 | 5800 | M7 | Other | NE | RL | 8 | No | No | 9 | Leukemia |
5 | F | 1 | 18 000 | M7 | −7+ring/marker | NE | RL | 12 | Yes | UCBT | 16 | Leukemia |
6 | M | 0.7 | 19 200 | M7 | Other | NE | RL | 8 | Yes | RBMT | 28 | TRM |
7 | M | 0.7 | 6500 | M7 | Other | mutated | RL | 3 | No | UCBT | 7 | TRM (non-CR) |
8 | F | 5 | 25 800 | M2 | t(8;21)(q22;q22) | NE | IF | 0 | Yes | UCBT | > 54‡ | |
9 | F | 1 | 4800 | M7 | Monosomy 7 | mutated | RL | 3 | No | RPBSCT | 10 | Leukemia |
10 | M | 14 | 1900 | M7 | Na | WT | RL | 17 | Yes | RBMT | 60 | TRM |
11§ | M | 1 | 11 600 | M7 | Monosomy 7 | NE | RL | 18 | Yes | No | 33 | Leukemia |
12 | M | 3 | 2600 | M7 | Constitutional | NE | RL | 3 | No | RBMT | 19 | Leukemia |
13 | M | 4 | 2800 | MDS | −7+ring/marker | NE | RL | 12 | Yes | UBMT | 11 | Leukemia |
14 | M | 15 | 1800 | RAEBt | Other | NE | IF | 2 | No | UCBT | 11 | Leukemia |
15 | F | 10 | 900 | M1 | Other | NE | RL | 39 | Yes | No | > 33 | |
16 | M | 3 | 6700 | M7 | Constitutional | mutated | RL | 72 | Yes | No | > 28 | |
17 | M | 1 | 5100 | M7 | Other | NE | RL | 10 | Yes | No | > 46 | |
18 | M | 2 | 5600 | MDS | −7+ring/marker | mutated | RL | 9 | No | No | 10 | Leukemia |
19 | M | 1 | 7400 | M7 | Constitutional | mutated | RL | 7 | No | No | 3 | Leukemia |
20 | M | 1 | 143 600 | M7 | Constitutional | NE | RL | 11 | Yes | RBMT | > 84 | |
21 | M | 2 | 8600 | M7 | Other | mutated | RL | 5 | No | No | 4 | Leukemia |
22 | F | 3 | 5900 | M7 | Other | NE | RL | 11 | Yes | UBMT | > 32 | |
23 | M | 16 | 38 800 | M5a | Other | NE | RL | 20 | Yes | No | > 23 | |
24 | M | 1 | 3480 | M7 | Other | NE | RL | 9 | Yes | UCBT | 14 | Leukemia |
25 | F | 3 | 2100 | MDS | Other | mutated | RL | 7 | No | No | 3 | Leukemia |
26 | F | 2 | 2900 | M7 | −7+ring/marker | NE | RL | 11 | NA | Yes | 11 | unknown |
27 | F | 2 | 2700 | M7 | Other | mutated | RL | 4 | No | No | 4 | Leukemia |
28 | M | 1 | 2470 | M7 | −7+ring/marker | NE | RL | 8 | No | No | 4 | Leukemia |
29 | F | 1 | 3680 | M7 | Na | NE | RL | 4 | No | UCBT | 5 | Leukemia |
No. . | Clinical characteristics at initial diagnosis of ML-DS . | GATA1 status . | IF/RL . | Time to IF/RL, mo* . | Subsequent CR . | HSCT . | Survival, mo† . | Cause of death . | ||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
Sex . | Age, y . | WBC, /μL . | FAB . | Karyotype . | ||||||||
1 | F | 3 | 3000 | M7 | Constitutional | NE | RL | 4 | No | No | 5 | Leukemia |
2 | M | 3 | 51 700 | M7 | Constitutional | NE | IF | 1 | No | UCBT | 8 | Leukemia |
3 | M | 2 | 10 000 | M7 | Other | NE | RL | 2 | No | No | 3 | Leukemia |
4 | F | 2 | 5800 | M7 | Other | NE | RL | 8 | No | No | 9 | Leukemia |
5 | F | 1 | 18 000 | M7 | −7+ring/marker | NE | RL | 12 | Yes | UCBT | 16 | Leukemia |
6 | M | 0.7 | 19 200 | M7 | Other | NE | RL | 8 | Yes | RBMT | 28 | TRM |
7 | M | 0.7 | 6500 | M7 | Other | mutated | RL | 3 | No | UCBT | 7 | TRM (non-CR) |
8 | F | 5 | 25 800 | M2 | t(8;21)(q22;q22) | NE | IF | 0 | Yes | UCBT | > 54‡ | |
9 | F | 1 | 4800 | M7 | Monosomy 7 | mutated | RL | 3 | No | RPBSCT | 10 | Leukemia |
10 | M | 14 | 1900 | M7 | Na | WT | RL | 17 | Yes | RBMT | 60 | TRM |
11§ | M | 1 | 11 600 | M7 | Monosomy 7 | NE | RL | 18 | Yes | No | 33 | Leukemia |
12 | M | 3 | 2600 | M7 | Constitutional | NE | RL | 3 | No | RBMT | 19 | Leukemia |
13 | M | 4 | 2800 | MDS | −7+ring/marker | NE | RL | 12 | Yes | UBMT | 11 | Leukemia |
14 | M | 15 | 1800 | RAEBt | Other | NE | IF | 2 | No | UCBT | 11 | Leukemia |
15 | F | 10 | 900 | M1 | Other | NE | RL | 39 | Yes | No | > 33 | |
16 | M | 3 | 6700 | M7 | Constitutional | mutated | RL | 72 | Yes | No | > 28 | |
17 | M | 1 | 5100 | M7 | Other | NE | RL | 10 | Yes | No | > 46 | |
18 | M | 2 | 5600 | MDS | −7+ring/marker | mutated | RL | 9 | No | No | 10 | Leukemia |
19 | M | 1 | 7400 | M7 | Constitutional | mutated | RL | 7 | No | No | 3 | Leukemia |
20 | M | 1 | 143 600 | M7 | Constitutional | NE | RL | 11 | Yes | RBMT | > 84 | |
21 | M | 2 | 8600 | M7 | Other | mutated | RL | 5 | No | No | 4 | Leukemia |
22 | F | 3 | 5900 | M7 | Other | NE | RL | 11 | Yes | UBMT | > 32 | |
23 | M | 16 | 38 800 | M5a | Other | NE | RL | 20 | Yes | No | > 23 | |
24 | M | 1 | 3480 | M7 | Other | NE | RL | 9 | Yes | UCBT | 14 | Leukemia |
25 | F | 3 | 2100 | MDS | Other | mutated | RL | 7 | No | No | 3 | Leukemia |
26 | F | 2 | 2900 | M7 | −7+ring/marker | NE | RL | 11 | NA | Yes | 11 | unknown |
27 | F | 2 | 2700 | M7 | Other | mutated | RL | 4 | No | No | 4 | Leukemia |
28 | M | 1 | 2470 | M7 | −7+ring/marker | NE | RL | 8 | No | No | 4 | Leukemia |
29 | F | 1 | 3680 | M7 | Na | NE | RL | 4 | No | UCBT | 5 | Leukemia |
CR indicates complete response; F, female; IF, induction failure; M, male; NA, data not available; NE, not evaluated; other, other cytogenetic abnormalities; RBMT, related bone marrow transplantation; RPBSCT, related peripheral blood stem cell transplantation; RL, relapse; TRM, transplantation-related mortality; UBMT, unrelated bone marrow transplantation; and UCBT, unrelated cord blood transplantation
Months from initial diagnosis to either induction failure or first relapse.
Duration of survival from induction failure or first relapse.
Patient no. 8 is alive with disease.
Patient no. 11 underwent HSCT in third CR on day 981 after relapse and died of transplantation-related toxicity on day 981.