Clinical features of children with congenital leukaemia and transient myeloproliferative disorder
| Patient | Year of diagnosis | Age in days (sex) | Clinical phenotype | Skin involvement | White cell count ×109/l (% blasts) | Bone marrow blasts (%) | Immunphenotype | Karyotype | Specific treatment | Outcome | Diagnosis |
|---|---|---|---|---|---|---|---|---|---|---|---|
| ALL, acute lymphoblastic leukaemia; AML, acute myeloid leukaemia; DAT, daunorubicin, cytarabine, thioguanine; RSV, respiratory syncytial virus; TdT, terminal deoxynucleotidyl transferase; TMD, transient myeloproliferative disorder; UD BMT, unrelated donor bone marrow transplant. | |||||||||||
| 1 | 1984 | 42 (F) | Normal | nodules | 66 (7) | 30 | Not done | 46 XX | DAT | Died age 46 days | AML M4 |
| 2 | 1994 | 0 (F) | Down | No | 119 (100) | Not done | Not done | Not done | None | Died age 1 day | AML M7 |
| 3 | 1994 | 17 (M) | Down | No | 9 (2) | 12 | Not done | 47 XY, +21 | None | Recovered spontaneously | TMD |
| 4 | 1995 | 0 (M) | Normal | No | 39 (90) | Not done | Not done | 46 XY 47 XY, +21 | None | Recovered spontaneously. No features of Down’s syndrome | TMD |
| 5 | 1996 | 88 (F) | Normal | No | 50 (100) | 95 | CD19, HLADR, TdT positive CD10/13/33/3/7 negative | 46 XX, t(4;11) | Infant ALL regimen | Remission UD BMT Relapse Died age 2 years | Null ALL |
| 6 | 1996 | 70 (F) | Down | No | 6 (1) | 15 | CD13/33/34/7 positive CD10/19/3, TdT negative | 47 XX, +21 | None DAT for relapse | Recovered Relapsed AML Remission persists | TMD and AML |
| 7 | 1997 | 8 (M) | Down | No | 90 (100) | 90 | Not done | 46 XY | Cytarabine 5mg | Blast count fell | TMD |
| 47 XY, +21 | Cytarabine 7.5 mg as single doses | Complete recovery | |||||||||
| 8 | 1997 | 0 (F) | Normal | No | 354 (100) | 90 | CD13/33 positive CD3/10/19/61 negative | 46 X, ins(X;6) | Cytarabine 7.5 mg as single dose | Remission persists | AML M0 ?Null ALL |
| Hybrid ALL and AML regimen UD BMT | |||||||||||
| 9 | 1999 | 54 (M) | Normal | No | 100 (100) | Not done | CD19 positive CD10/3/13/33, Tdt negative | 46 XY, t(11;19) | Infant ALL regimen | Remission RSV pneumonitis Died age 1 year | Null ALL |