Abstract Ref Number = APCP897
JUVENILE CHRONIC MYEOBLASTIC LEUKEMIA WITH SHORT STATURE, OSTEOPOROSIS, AND SEVERE MALNUTRITION FOUR YEARS FOLLOW UP CASE REPORT
Wira Mondana,Bidasari Lubis,Olga Rasiyanti Siregar,Siska Mayasari Lubis,Tiangsa Sembiring
Department of Child Health Faculty of Medicine Universitas Sumatera Utara
Background : Juvenile chronic myeloblastic leukemia (JCML) is a rare malignant hematopoietic disorder representing only 2% of all childhood leukemia. The basic diagnosis is the presence of translocation of chromosome known as Philadelphia chromosome detected by PCR. Malnutrition during treatment for childhood cancer not only has substantial clinical implications but may also adversely affect a child’s quality of life. Therapies such as corticosteroids, cytotoxic and radiotherapy will have harmful effect on bone mineral density and long-term musculoskeletal effects of chemotherapy in cancers may include avascular necrosis and can increase the possibility of osteoporosis and pathological fractures due to loss of bone density.
Case Presentation Summary : A 2 years old female came with complaints of pale. No history of spontaneous bleeding. Fever suffered by patient 3 weeks before hospital admission. Progressive abdominal distension were found. Hepatomegaly palpable at 4 cm below the arcus costae and spleen palpable at schuffner VII. Nutritional status and height was below at <5th percentile based on WHO charts. On the day of admission the following investigations were ordered: complete blood count with peripheral smear analysis, comprehensive chemistry pannel, chest X-ray, ECHO, bone marrow punction. In a complete blood laboratory investigation, Hb:7.4 gr %, Leucocyte:342.320/mm3, Platelet:459.000/mm3. The result of bone marrow punction: bone marrow indicates the dominance of myeloblast pathologic with complete maturation and other systems urged. A bone age study revealed growth failure of her metacarpals and phalanges and showed osteoporotic bone. The translocation events involving the M-bcr transcripts b3a2 transcript detected (p210/Major breakpoint cluster region). Imatinib is a potent inhibitor of the ABL tyrosine kinase.
Learning Points/Discussion : JCML with osteoporosis, severe malnutrition, and short stature is rare. Imatinib has proven to work on decreasing the production of white blood cells from the hemopoietic stem system. A good patient follow up mechanism and documentation of patient information needs to be put in place in health facilities so that to be able to get treatment outcomes of the medical management.
Keywords: leukemia malnutrition osteoporosis short stature