Table 3.
Cancer in the Major IBMFS
E) Shwachman-Diamond Syndrome
| Type | Subtype | Number* | Male | Female | M:F | Median Age, Yrs (Range) |
|---|---|---|---|---|---|---|
| Leukemia | Total leukemias | 38 | 26 | 9 | 2.89 | 19 (2–43) |
| AML | 31 | 19 | 9 | 21 (3–43) | ||
| ALL | 5 | 5 | 0 | 14 (2–14) | ||
| JCML | 1 | 1 | 0 | 8 | ||
| AML after BMT | 1 | 1 | 0 | 21 | ||
| MDS | Total MDS | 72 | 47 | 23 | 2.04 | 8 (0.5–42) |
| RA | 36 | 23 | 13 | 8 (0.8–35) | ||
| RARS | 1 | 1 | 0 | 23 | ||
| RAEB | 5 | 5 | 0 | 19 (4–30) | ||
| RAEBT | 3 | 3 | 0 | 6, 8, 42 | ||
| Clone alone | 25 | 15 | 10 | 10 (0.5–25) |
*
11 patients had MDS prior to leukemia. One of these had RA pre-BMT, followed by AML post-BMT. 25 patients are included as MDS if they had only a clone without MDS morphology. Gender was missing in 3 cases. JCML, juvenile chronic myelogenous leukemia. BMT, bone marrow transplant. RA, refractory anemia. RARS, refractory anemia with ringed sideroblasts. RAEB, refractory anemia with excess blasts. RAEBT, refractory anemia with excess blasts in transformation.