Table 6.
Overall outcomes for patients with severe chronic neutropenia
| Congenital | Cyclic | Autoimmune/Idiopathic | Total | |
|---|---|---|---|---|
| Patients | 670 | 266 | 816 | 1752 |
| AML/MDS | 76 (11.3%) | 1 (0.01%) | 0 | 77 |
| HSCT, n (%) | 127 (AML: 58; neutropenia: 68, CML: 1) (19.0%) |
4 (AML: 1; neutropenia: 3) (1.9%) |
6 (AML: 0; neutropenia: 5; CMML: 1) (1.0%) |
137 |
| Causes of death | 92 | 24 | 43 | 159 |
| Infection | 21 | 7 | 8 | 36 |
| HSCT, not AML related | 11 | 2 | 1 | 14 |
| AML with HSCT | 35 | 1 | 0 | 36 |
| AML without HSCT | 16 | 1 | 0 | 17 |
| Accident | 0 | 2 | 0 | 2 |
| LPDs | 3 | 0 | 5 | 8 |
| Cardiac | 0 | 2 | 5 | 7 |
| Unknown | 4 | 3 | 12 | 19 |
| Nonmyeloid malignancies (cancer) | 1 (renal) |
2 (1 colon, 1 hepatic) |
4 (2 colon, 1 hepatic, 1 lung) |
7 |
| Other | 1 | 4 | 8 | 13 |
CML, chronic myeloid leukemia; CMML, chronic myelomonocytic leukemia; LPD, lymphoproliferative disease.