2. CASE DESCRIPTION
Case 1: ARA is a 1 year 5 months old girl, admitted to the hospital looking pale and with fever. Physical findings were that of anemia and hepatosplenomegaly. Blood counts showed hemoglobin of 4.4 g/dL, leucocytes of 51,840/mm3, and platelets of 5,000/mm3. The peripheral blood smear showed domination of lymphoblast cell (71%), the lymphocyte size was big, without any granule, and no Auer rods appearance, with impression of Acute Lymphoblastic Leukemia (ALL) and the bone marrow aspiration showed a 26% blast with morphology of ALL. The immunophenotyping was positive for the myeloid lineage. DNA analysis for GATA-1 mutation is not available due to laboratory issue. She was treated using ML-DS protocol, consisting of triple intrathecal (methotrexate, dexamethasone, and ara-C) on day 1, vincristine (1.5 mg/m2 i.v, max 2 mg) on day 1 and day 8, daunorubicin (50 mg/m2/day i.v) on day 8, and cytarabine (100 mg/m2/dose i.v pushed twice a day) on day (see figure 1) 8 to 12. The course was given twice, with an interval of 3 to 4 weeks (Figure 1). Upon diagnosis the blood count profile was hemoglobin 13.3 g/dL, leucocytes of 10.730/mm3, and platelets 241.000/mm3. The leucocytes levels on day 8th of treatment was 4.407/mm3. She experienced febrile neutropenia once and delayed in chemotherapy schedule. Till date, she is surviving for 46 months.
Case 2: MBC is a 1 year 9 months old girl, admitted to the hospital looking pale and with fever. Physical findings were that of anemia, pansystolic murmur, and hepatosplenomegaly. Blood counts showed hemoglobin of 2.8 g/dL, leucocytes of 4,500/mm3 and platelets of 10,000/mm3. Echocardiography showed an atrial septal defect. The initial blood smear shown an relative lymphocytosis. On bone marrow aspiration showed 75.5% myeloblast with morphology of AML-M2. The immunophenotyping was positive for the myeloid lineage of the blasts. DNA analysis was positive for GATA-1. She was treated using the same protocol, initial laboratory before the start of chemotherapy was hemoglobin 7.6 g/dl, leucocyte 7.471 /mm3, platelets 10.000 /mm3. On day 8th of chemotherapy, the leucocyte level was 2.399 /mm3. Along the course of treatment, the patient experienced one episode of severe febrile neutropenia, and several times of severe thrombocytopenia. However, the patient died because of uncontrolled bleeding two days after the completion of therapy.
Case 3: AKY is a 2 years and 2 months old boy, admitted to hospital with fever. The physical finding was that of hepatosplenomegaly. Blood counts showed hemoglobin of 11.9 g/dL, leucocytes of 8,640/mm3, and platelets of 2,000/mm3. The peripheral blood smear shown a 33% myeloblasts without maturation, with morphology of AML-M5a, and the bone marrow aspiration shown 76% of myeloblasts with characteristics of AML-M5b. The immunophenotyping was positive for myeloid lineage. Cytogenetics showed Trisomy 21. The DNA analysis for GATA-1 mutation was positive. He was treated using the same AML-DS Protocol. Upon admission the hemoglobin was 8.8 g/dl, leucocyte 88.800 /mm3, platelets of 13.000 /mm3, on day 8 blood counts profile was Hb 11.1 g/dl, leucocyte 83.000 /ul, platelets of 20.000/ul. The patient experienced febrile neutropenia twice, severe thrombocytopenia once, and malaria infection. Until now, he is surviving for 30 months.
Along the course of treatment, all of the patients receive a supportive transfusion of packed-red cells and thrombocyte. Bacterial and fungal infection prophylaxis was given with amoxicillin-clavulanic acid and ketoconazole. The total cumulative doses for cytarabine is 2000 mg/m2, daunorubicin is 40 mg/m2 and vincristine is 6 mg/m2.