ABSTRACT
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.
ABSTRACT
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.
