Juvenile Myelomonocytic Leukemia in a Child: A Case Report of Palliative Chemotherapy and Literature Review Applied to Limited Resources Centers.

Juvenile myelomonocytic leukemia (JMML) is a rare hematopoietic malignancy in children, with an incidence of 1.2 per million children per year. At this moment, we present a case report and a brief literature review of JMML in a child, primarily focused on its applicability in low-middle income countries. A 3.5-year-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 hemoglobin of 6.4 g/dl, white blood cell of 315.62 × 103/µL, and platelet of 17 × 103/µL. Blood smears showed 10% suspected blasts, 17% myelocytes, and 17% metamyelocytes with thrombocytopenic crisis. The HbF level was 5.8%. BCR-ABL gene tested negative. The patient was diagnosed with juvenile myelomonocytic leukemia. Considering that HSCT could not be done in our center and lack other financial possibilities to seek treatment abroad, the family agreed to do the palliative treatment. The patient was treated with oral 6-mercaptopurine and subcutaneous cytarabine. Four weeks after receiving 6-mercaptopurine, the white blood cell count decreased to 10.6 × 103/µL and the spleen size was half of the original size. The patient continued chemotherapy until week 15, chemotherapy was stopped, but 16 weeks after the diagnosis of JMML, he developed severe thrombocytopenia, endophthalmitis, and sepsis and passed away. As a conclusion, in JMML cases in developing countries without HSCT, palliative chemotherapy is acceptable, and palliative care is an important aspect.

Plasmodium falciparum found in the bone marrow of a child in Manado City, East Indonesia: A case report.

In Indonesia, there are at least 1.3 million cases of malaria each year and Plasmodium falciparum appears to be the most common Plasmodium. The finding of Plasmodium is important for the diagnosis and management of malaria. This is a case of a 4-year-and-9-month-old male who lived in Manado, East Indonesia. He presented with a prolonged fever, was pale in appearance, and was easily fatigued over the last 3 weeks. Hepato-splenomegaly was found on the initial physical examination. Preliminary laboratory findings found pancytopenia and severe anemia. Before he was referred to our hospital, at the primary health center, the initial work-up was negative for Plasmodium with the serial Rapid Diagnostic Test and microscopic peripheral blood smears. Since there were signs and symptoms mimicking malignancy, the patient was referred to our hospital for further malignancy work-up. A bone marrow puncture was done and we incidentally found Plasmodium falciparum in a microscopic bone marrow smear. This was a rare case because Plasmodium was not initially found in the preliminary work-up (Rapid Diagnostic Test and Microscopic) and qPCR is not a routine work-up for Plasmodium suspected patients. Although the mortality rate of malaria is high, this condition can be treated if the clinician was aware of the clinical signs and symptoms in the early onset and prompt medical treatment is administered. In a severe case with an unclear etiology of fever and with signs and symptoms mimicking malignancy, qPCR is recommended. However, a bone marrow puncture can also be considered to exclude the possibility of a malaria infection.

Parental Experiences with Chemotherapy-Induced Alopecia among Childhood Cancer Patients in Indonesia.

BACKGROUND: This study assessed parental experiences with chemotherapy-induced alopecia among children with cancer treated at an Indonesian academic hospital. MATERIALS AND METHODS: Fifty parents of childhood cancer patients were interviewed using semi-structured questionnaires. RESULTS: The moment that hair fell out was the moment that parents (84%) had to admit their child had cancer. Alopecia was a traumatizing painful experience (46%). Active strategies to hide alopecia, mainly hats, were used by 66% of children, while 34% never covered their bald head. If money had not been an issue, 40% would use another strategy. Alopecia made children limit outdoor daily activities (78%) and engagement with others (60%). Significantly more children from high-educated (95%) than low-educated (60%) parents received sympathy from other people (P=0.012). Significantly more Christian (29%) than Muslim (0%) families confirmed that alopecia lowered the quality of life (P=0.046). Most parents (82%) had no prior plans about alopecia management, yet for significantly more girls (26%) than boys (0%) such plans existed (P=0.044). Parents received most information about alopecia from other parents (66%). Parents (92%) needed more alopecia education from doctors. Of all school-attending children, 53% were bullied and 47% did not want to attend school due to alopecia. Significantly more high-educated than low-educated families received pity from teachers and pupils (94% vs. 0%, P=0.004), and acceptance by pupils (81% vs. 0%, P=0.021). CONCLUSIONS: Alopecia is a severe, far-stretching side-effect of chemotherapy with physical, psychological and social consequences for children and parents. Parents should be better informed about occurrence and impact of alopecia. Extra attention is required to facilitate children's return to school. Health- care providers should facilitate optimal supportive care through open dialogue and provision of educational materials for parents, children and their community.

Comparing Health-Care Providers' Perspectives on Complementary and Alternative Medicine in Childhood Cancer Between Netherlands and Indonesia.

BACKGROUND: Use of complementary and alternative medicine (CAM) is common among patients with childhood cancer. Health-care providers (HCP) should address this need properly. Geographical and cultural differences seem likely. This study explores perspectives on CAM of HCP involved in the care of children with cancer in Netherlands and Indonesia. Health beliefs, components of CAM, encouraging or discouraging CAM, and knowledge about CAM were assessed. PROCEDURE: We conducted a cross-sectional study using semi-structured questionnaires at a Dutch and Indonesian academic hospital. RESULTS: A total of 342 HCP participated: 119 Dutch (response rate 80%) and 223 Indonesian (response rate 87%). Chemotherapy can cure cancer according to more Dutch than Indonesian HCP (87% vs. 53% respectively, P < 0.001). Combination of chemotherapy and CAM is the best way to cure cancer according to more Indonesian than Dutch HCP (45% vs. 25%, P < 0.001). Dutch and Indonesian HCP recommend and discourage CAM use differently. Most Dutch (77%) and Indonesian HCP (84%) consider their knowledge about CAM to be inadequate (P = ns). Fewer Dutch doctors than other HCP want to learn more about CAM (51% vs. 76%, P = 0.007), whereas there is no significant difference in eagerness to learn about CAM between Indonesian doctors (64%) and other HCP (72%). CONCLUSIONS: Indonesian HCP have more positive views about CAM than their Dutch colleagues. Both Dutch and Indonesian HCP consider their knowledge about CAM to be inadequate. Therefore, education programs about CAM tailored to the needs of HCP are recommended, knowing that CAM is used frequently.

Parents' and health-care providers' perspectives on side- effects of childhood cancer treatment in Indonesia.

BACKGROUND: Efficacy of childhood cancer treatment in low-income countries may be impacted by parents' and health-care providers' perspectives on chemotherapy-related side-effects. This study explores prevalence and severity of side-effects in childhood cancer, and compares health beliefs about side-effects between parents and health-care providers, and between nurses and doctors in Indonesia. MATERIALS AND METHODS: Semi-structured questionnaires were filled in by 40 parents and 207 health-care providers in an academic hospital. RESULTS: Parents exporessed a desire to receive more information about side-effects (98%) and worried about this aspect of treatment (90%), although side-effects were less severe than expected (66%). The most frequent was behavior alteration (98%) and the most severe was hair loss. Only 26% of parents consulted doctors about side-effects. More parents, compared to health-care providers, believed that medicines work better when side-effects are more severe (p<0.001), and accepted severe side-effects (p=0.021). More health-care providers, compared to parents, believed that chemotherapy can be stopped or the dosage altered when there are side-effects (p=0.011). More nurses, compared to doctors, stated that side-effects were unbearable (p=0.004) and made them doubt efficacy of treatment (p<0.001). CONCLUSIONS: Behavior alteration is the most frequent and hair loss the most severe side-effect. Apparent discrepancies in health beliefs about side-effects exist between parents and health-care providers. A sustainable parental education program about side-effects is recommended. Health-care providers need to update and improve their knowledge and communication skills in order to give appropriate information. Such measures may improve outcome of childhood cancer treatment in low-income countries, where adherence to therapy is a major issue.

Socio-economic status plays important roles in childhood cancer treatment outcome in Indonesia.

BACKGROUND: The influence of parental socio-economic status on childhood cancer treatment outcome in low-income countries has not been sufficiently investigated. Our study examined this influence and explored parental experiences during cancer treatment of their children in an Indonesian academic hospital. MATERIALS AND METHODS: Medical charts of 145 children diagnosed with cancer between 1999 and 2009 were reviewed retrospectively. From October 2011 until January 2012, 40 caretakers were interviewed using semi-structured questionnaires. RESULTS: Of all patients, 48% abandoned treatment, 34% experienced death, 9% had progressive/ relapsed disease, and 9% overall event-free survival. Prosperous patients had better treatment outcome than poor patients (P<0.0001). Odds-ratio for treatment abandonment was 3.3 (95%CI: 1.4-8.1, p=0.006) for poor versus prosperous patients. Parents often believed that their child's health was beyond doctor control and determined by luck, fate or God (55%). Causes of cancer were thought to be destiny (35%) or God's punishment (23%). Alternative treatment could (18%) or might (50%) cure cancer. Most parents (95%) would like more information about cancer and treatment. More contact with doctors was desired (98%). Income decreased during treatment (55%). Parents lost employment (48% fathers, 10% mothers), most of whom stated this loss was caused by their child's cancer (84% fathers, 100% mothers). Loss of income led to financial difficulties (63%) and debts (55%). CONCLUSIONS: Treatment abandonment was most important reason for treatment failure. Treatment outcome was determined by parental socio-economic status. Childhood cancer survival could improve if financial constraints and provision of information and guidance are better addressed.

Immunophenotypic patterns of childhood acute leukemias in Indonesia.

BACKGROUND: Immunophenotyping, as suggested by WHO, may improve diagnosis of childhood leukemia since it offers a better classification of the hematopoietic lineage of malignant cells as compared to morphology. Therefore, we aimed to determine the proportion of the immunophenotypic subtypes of acute leukemia in Indonesian children. METHODS: Samples were obtained from patients (0-14 years of age) in 4 hospitals in Indonesia. We analyzed 541 suspected leukemia samples presented over a 4-year period (March 2006 - July 2010) by flow cytometry. Immunophenotyping allowed classification into acute myeloid leukemia (AML) and ALL (B-lineage and T-lineage ALL). RESULTS: Of 541 samples, 136 were tested using a single color method and 405 with a three-color method. Concordance with morphology was very good (?=0.82) using the three-color method with a panel of 15 monoclonal antibodies (n=387). A relatively high percentage of acute leukemia was classified as AML (23%). Of the ALL samples 83% were B-lineage ALL and 17% T- lineage ALL. Nine out of 239 morphological ALL were labeled AML, and 12/79 morphological AML were in fact ALL. CONCLUSION: Immunophenotyping in a multi-center study proved feasible and appears particularly important for prognostic assessment of childhood leukemia in low income countries such as Indonesia.

Varicella outbreak in a pediatric oncology ward: the Manado experience.

BACKGROUND: Varicella is highly contagious and dangerous disease, especially in immunocompromised patients. Children with cancer are at increased risk of severe illness and fatal cases occur. OBJECTIVE: To describe an outbreak of varicella among in-patient cancer children, family members and staff. Estella Children Cancer Center in Manado, Indonesia with 14 beds and a 15 bed capacity guest house for family members. METHODS: A retrospective study of patients, family members and staff who were diagnosed with varicella based on clinical appearance was performed. Follow up was until 28 days from the last patient diagnosis' date. RESULTS: From late February to early May 2009, varicella was affecting 4 among 8 children with leukemia, 1 family member and 1 housekeeping staff. Measurers taken after the index case were oral acyclovir both for patients and contacts, patient isolation, ward disinfection and some chemotherapy interruption. Nevertheless, a second and third wave of varicella occurred. The index case died due to encephalitis. Other patients were non-severe and cured, but one child was lost to follow up. CONCLUSIONS: This outbreak highlights the importance of proper prevention and prompt management of varicella in immunocompromised patients. Simple and locally applicable guidelines are needed.