Juvenile myelomonocytic leukemia in a child: a case report of palliative
chemotherapy and literature review applied to limited resources centers
Abstract
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.