Successful Treatment of Acute Lymphoblastic Leukemia in a Child with Trisomy 21 and Complex Congenital Heart Disease with Mechanical Prosthetic Valve

Table 1

Modified chemotherapy regimen used for our subject using the standard-risk ALL0331 backbone with modifications.

Chemotherapeutic drug	Route	Dose	Days
Phase i: induction
Intrathecal ara-C	IT	70 mgs	Day 1
^*Intrathecal methotrexate	IT	15 mgs	Day 8, 29
Vincristine	IV	1.5 mgs/m^2	Day 1, 8, 15, 22
PEG-asparaginase	IM	1500 iu/m^2	Day 4
^**Prednisone	PO	60 mg/m^2	Day 1–28
^**PO Prednisone as per CCG 1961 added instead of dexamethasone.
Phase ii: standard consolidation
^*Intrathecal methotrexate	IT	15 mgs	Day 1, 8, 15
Vincristine	IV	1.5 mgs/m^2	Day 1
6-Mercaptopurine	PO	75 mgs/m^2	Day 1–28
Phase iii: standard interim maintenance
Dexamethasone	PO	6 mgs/m^2	Days 1–5
			Days 29–33
Vincristine	IV	1.5 mgs/m^2	Days 1 and 29
6-Mercaptopurine	PO	75 mgs/m^2	Days 1–50
^*Intrathecal methotrexate	IT	15 mgs	Day 29
Phase iv: augmented delayed intensification
Vincristine	IV	1.5 mgs/m^2	Days 1, 8, 15, 43, 50
PEG-asparaginase	IM	1500 iu/m^2	Days 4 or 5 or 6 and 43
Cyclophosphamide	IV	1000 mgs/m^2	Day 29
Cytarabine	IV	75 mgs/m^2	Days 29–32
			Days 36–39
Thioguanine	PO	60 mgs/m^2	Days 29–42
^*Intrathecal methotrexate	IT	15 mgs	Day 8, 29, 43
Anthracyclines (doxorubicin) removed from the protocol due to the underlying cardiac dysfunction.
Phase v: maintenance (total 8 cycles each of 84 days)
Intrathecal methotrexate	IT	15 mgs	Day 1 of cycle 1 and 2
6-Mercaptopurine	PO	75 mgs/m^2	Day 1–84
Methotrexate	PO	20 mgs/m^2	weekly
Vincristine	IV	1.5 mgs/m^2	Day 1, 29, 57
Dexamethasone	PO	6 mgs/m^2	5 days every 4 weeks days 1–5, 29–33, 57–61

^*Leucovorin PO 5 mg/m² every 12 hrs 2 doses 48 and 60 hrs after each IT.
Note: IT MTX doses are age adjusted. All intrathecals were administered under fluoroscopic guidance.