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.