| ICD-Topo |
C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS |
| ICD-Morpho |
9920/3 Therapy-related myeloid neoplasms
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| Atlas_Id |
1296 |
| Note |
This data is extracted from a very large study from an International Workshop on treatment related leukemias - restricted to balanced chromosome aberrations (i.e.: -5/del(5q)and -7/del(7q) not taken into account per see), published in Genes, Chromosomes and Cancer in 2002. |
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t(8;21)(q22;q22) RUNX1/RUNX1T1 . Fluorescence in situ hybridization with the Vysis LSI RUNX1/RUNX1T1 dual color dual fusion probes (Abbott Molecular, US) showing 2 copies of genes on normal (A) and fusion red-green signals on der(8) and der(21) chromosomes, the most frequently encountered FISH pattern in patients with t(8;21)(q22;q22) (B). Variations in the FISH patterns include the presence of 1 fusion signal on der(8) chromosome (C), insertion of RUNX1T1 into chromosome 21 (D) and cryptic fusion gene on der(21) with normal appearing chromosomes (E). Courtesy Adriana Zamecnikova. |
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| Disease |
Treatment related myelodysplasia (t-MDS) or acute non lymphocytic leukaemias (t-AML) |
| Note |
The study included 79 cases; t-MDS without progression to AML accounted for 15%, t-MDS progressing to AML for 18%, t-AML for the remaining 67%; there was no case of acute lymphoblastic leukaemia |
| Phenotype / cell stem origin |
MDS cases were frequently refractory anemia with excess of blasts cases; 58% of AML cases were M2 AML |
| Etiology | Frequent antracyclin exposure |
| Epidemiology | 21q22 rearrangements were found in 15% of t-MDS/t-AML; 1M to 1F sex ratio |
| Clinics | Age at diagnosis of the primary disease was 47 yrs (range 2-75); age at diagnosis of the t-MDS/t-AML was 51 yrs (11-77) and median interval was 39 mths (6-306). Primary disease was a solid tumor in 56% of cases (mainly: breast, lung, sarcoma/ PNET, colon cancer) and an hematologic malignancy in 43%. Treatment of the primary disease included radiotherapy (in 6%), chemotherapy (46%) or both (48%). 75% of patients with a 21q22 rearrangement had previously received topoisomerase II inhibitors, a higher proportion than other subgroups of treatment related leukemia, except 11q23 patients, who were 84% to have been exposed to topoisomerase II inhibitors; alkylating agents exposure was higher than in patients with t(15;17) or inv(16) |
| Treatment | Patients who received bone marrow transplantation had a higher median survival (31 mths). |
| Prognosis | Median survival was 14 mths, there was 58% of patients surviving 1 yr, 33% 2 yrs, and 18% 5 yrs., a better outcome than patients with 11q23 rearrangement, 3q21q26 rearrangement, 12p13 rearrangement, t(9;22), or t(8;16) and a worse outcome than those with t(15;17) or inv(16) treatment related leukemias.By th 21q22 group, patients with a t(8;21) had a better outcome, and those with a t(3;21) had a worse outcome. |
