Differences in MDSs between children and adults
| . | Children (0-18 y) . | Adults (older than age 40 y) . |
|---|---|---|
| Incidence per million | 1-4 | >40 |
| Refractory anemia with ringed sideroblasts (%) | <1 | 25 |
| Associated IBMFSs and predisposition syndromes (%) | >30 | <5 |
| Familial aggregation | Present in a proportion of patients | Uncommon |
| Chromosomal aberrations (%) | ||
| −7/7q− | 25-30 | 10 |
| −5/5q− | 1 | 20 |
| Molecular aberrations | Presence of germ line mutations (eg, GATA2); less frequent somatic mutations; absent or exceptional spliceosomal mutations | Germ line mutations are less common; frequent somatic mutations; spliceosomal mutations are common |
| General aim of treatment | Curative | Often palliative |
| . | Children (0-18 y) . | Adults (older than age 40 y) . |
|---|---|---|
| Incidence per million | 1-4 | >40 |
| Refractory anemia with ringed sideroblasts (%) | <1 | 25 |
| Associated IBMFSs and predisposition syndromes (%) | >30 | <5 |
| Familial aggregation | Present in a proportion of patients | Uncommon |
| Chromosomal aberrations (%) | ||
| −7/7q− | 25-30 | 10 |
| −5/5q− | 1 | 20 |
| Molecular aberrations | Presence of germ line mutations (eg, GATA2); less frequent somatic mutations; absent or exceptional spliceosomal mutations | Germ line mutations are less common; frequent somatic mutations; spliceosomal mutations are common |
| General aim of treatment | Curative | Often palliative |
Adapted with substantial modifications from Hasle.5
IBMFS, inherited bone marrow failure syndrome.