Juvenile myelomonocytic leukemia (JMML) is a rare hematopoietic malignancy in children with incidence 1.2 per million children per year. Hereby we present a case report and brief literature review of JMML in a child, especially focused on applicability in low-middle income countries. A 3.5 years-old male was referred to our tertiary center due to pallor, enlarging abdomen and neck mass, recurrent fever and chronic diarrhea. Initial laboratory workup showed Hb 6.4 g/dl, white blood cell 315.62 x 103/uL, and platelet 17 x 103/uL. Blood smears showed 10% suspected blast, 17% myelocyte, 17% metamyelocytes, with thrombocytopenic crisis. The HbF level was 5.8%. BCR-ABL gene was tested negative. The patient was diagnosed as juvenile myelomonocytic leukemia. Considering that HSCT was not able to be done in our center and lack of financial possibilities to seek that treatment abroad, family agreed to do palliative treatment. Patient was treated with 6-mercaptopurin and subcutaneous cytarabine. Four weeks after receiving 6-MP, white blood cell count decreased to 10,6 x 103/uL and spleen size was half of the original size. Patient continued the chemotherapy until week 15, chemotherapy was stopped, but 16 weeks after the diagnosis of JMLL, he developed severe thrombocytopenia, endophthalmitis, sepsis and passed away.

Background: In 2001, Dr. Sardjito Hospital initiated a systematic hospital-based registry, Yogyakarta Pediatric Cancer Registry (YPCR). This study aims to present an epidemiological profile of childhood malignancies diagnosed in Dr. Sardjito General Hospital and compare it with the previous study 1 Methods: Childhood cancer was diagnosed in children aged 0-18 years, from January 2009 to December 2018, and analyzed. Childhood malignancies were categorized based on age, sex, and disease group according to the International Classification of Childhood Cancer (ICCC-3). An estimated annual average incidence rate (AAIR) of childhood cancer was calculated. We visualized the number of patients and their regions of origin by geographic mapping. Result: There were 1,788 new cases registered in YPCR during the study period. Of these, 58% were male, with a male-to-female.4:1.0. The mean age at diagnosis was 6.3 years old, the median age was 5 years and 56% of cancers were diagnosed in the age group of 0-5 years old. The most common diagnosis category was leukemia (ICCC-3 Category I), which accounted for 60% of all childhood malignancies. The three most common diagnoses included: ALL (44%), AML (13%), and retinoblastoma (6%). Of the 1,077 patients diagnosed with leukemia, 58% were males, most often diagnosed at 0-5 years old (53%). There were 679 patients registered with solid tumors mostly diagnosed at 0-5 years old (57%). The AAIR of leukemia and solid tumors was 26.8 and 17.5 per million, respectively. Conclusion: There was an increase in the number of childhood malignancies in 2009-2018 compared to the 2000-2009 study. The number of patients referred to our hospital increased, indicating a better referral system to the pediatric cancer center. This study is expected to provide data on the hospital-based pediatric cancer registry in Indonesia and promote systematic pediatric cancer registries in other centers.

Background: As in LMICs, the prognosis of childhood ALL in Indonesia was lower than in HICs. Indonesian-ALL2013 protocol resulted in more toxicities and abandonments than expected. Therefore, it was modified into a pilot ALL2016 protocol. Changes to the ALL2013 protocol: no anthracyclines in SR, dexamethasone replaced prednisone in reinduction for HR and some drugs were rescheduled. Procedure: We compare the outcome of ALL2013 and ALL2016. Results: A total of 383 children with ALL were diagnosed, 21 were excluded. ALL2013 included 174 patients (106 SR and 68 HR) and ALL2016 188 (91 SR and 97 HR). The outcome of the ALL2016 was better than the ALL2013 (pOS 67.0% vs 60.3%; p=0.087 and pEFS 50.0% vs 37.9%; p=0.012) even when the number of HR patients was significantly higher in ALL2016 (51.6% vs 39.1%). The ALL2016 showed an early advantage for SR patients (pEFS 56.7% vs 47.2%; p=0.114 and pOS 74.4% vs 69.8%; p=0.298) due to the decrease of toxic deaths (10.4% vs 5.5%; p=0.211) however the number of late relapses were still high (19.5% vs 13.2%; p=0.282). In the HR group, both pEFS and pOS were significantly better in ALL2016 (pEFS 43.3% vs 23.5%; p=0.010 and pOS 59.8% vs 45.6%; p=0.036) due to less relapses (14.4% vs 29.4%; p=0.019). Both SR and HR showed a smaller number of abandonments in ALL2016. Conclusions: After small changes in protocol, initial toxicity and abandonments were reduced and the pOS and pEFS were improved. However, relapses still need to be lessened in the next protocol.