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Juvenile Myelomonocytic Leukemia
Diagnosis
Patients with JMML may present with:
- Constitutional symptoms (eg, malaise, pallor, and fever) or evidence of an infection
- Symptoms of bronchitis or tonsillitis (in approximately 50% of cases)
- Bleeding diathesis
- Maculopapular skin rashes (in 40%-50% of cases)
- Lymphadenopathy (in approximately 75% of cases)
- Hepatosplenomegaly (in most cases)
A definitive diagnosis of JMML requires the following:
Major criteria (all 3 required)
- No Philadelphia chromosome or Bcr-Abl fusion gene
- Peripheral blood monocytosis >1 x 109/L
- <20% blasts (including promonocytes) in the blood and bone marrow
Minor criteria (2 or more required)
- Fetal hemoglobin (Hb F) increased for age
- Immature granulocytes in the peripheral blood
- White blood cell count >1 x 109/L
- Clonal chromosomal abnormality (eg, monosomy 7)
- Granulocyte-macrophage colony-stimulating factor (GM-CSF) hypersensitivity of myeloid progenitors in vitro
Reference:
National Cancer Institute. Myelodysplastic/Myeloproliferative Diseases (PDQ®): Treatment. Health Professional Version. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/mds-mpd/healthprofessional/allpages. Accessed April 24, 2006.
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