Erythroblastic and/or megakaryoblastic leukemia in Down syndrome: treatment with low-dose arabinosyl cytosine.

Abstract

PURPOSE:

We report here the clinical response to low-dose arabinosyl cytosine (Ara-C) in seven children with Down syndrome (DS) and acute leukemia in which blast cells express markers of erythroid and/or megakaryoblastic lineages. Following an initial course of treatment with Ara-C, complete remission was obtained in all seven patients. Maintenance therapy with Ara-C was continued during complete remission. Four patients subsequently relapsed; the three others are disease-free. Based on these data, we suggest that when conventional therapy is contraindicated by associated malformations, low-dose Ara-C can be used for treating DS patients with erythroblastic or megakaryoblastic leukemia. The aim of this study was to assess the efficacy of low-dose Ara-C in treating megakaryoblastic and/or erythroblastic leukemia associated with DS.

PATIENTS AND METHODS:

Seven patients with DS presented with leukemia in which blast cells displayed early markers of the erythroblastic and/or megakaryoblastic lineage. Low-dose subcutaneous Ara-C (10 mg/m2 two times per day) was given for 21 days as induction therapy, followed by a 5-10-day course each month for 2 years as a maintenance treatment.

RESULTS:

Low-dose Ara-C treatment resulted in complete remission in all seven patients and in long-term disease-free survival in three patients.

CONCLUSION:

In cases in which conventional chemotherapy is contraindicated, low-dose Ara-C should be considered as a therapeutic alternative for treatment of DS-associated erythroblastic or megakaryocytic leukemia.

• Authors: G Tchernia, F Lejeune, J F Boccara, M F Denavit, J P Dommergues, F Bernaudin
• Journal: Journal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 18 Issue 1 Pg. 59-62 (Feb 1996) ISSN: 1077-4114 [Print] United States
• PMID: 8556372 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antimetabolites, Antineoplastic
• Cytarabine

Topics

• Antimetabolites, Antineoplastic (administration & dosage, therapeutic use)
• Child, Preschool
• Cytarabine (administration & dosage, therapeutic use)
• Disease-Free Survival
• Down Syndrome (complications)
• Drug Administration Schedule
• Female
• Humans
• Infant
• Leukemia, Erythroblastic, Acute (complications, drug therapy)
• Leukemia, Megakaryoblastic, Acute (complications, drug therapy)
• Male
• Remission Induction