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Juvenile CML

  1. Children < yr
  2. Philadelphia chromosome is negative
  3. HbF is 15 – 50%
  4. Facial rash; bleeding manifestation and lymphadenopathy is frequent.
  5. Frequent normoblasts on peripheral smear.
  6. Poor response to Biosulphane.
  7. Median survival is 9 months.
Adult variety of CML Juvenile chronic myeloid leukemia
Age >4 yrs. <4 yrs. Patient with neurofibromatosis are at high risk for development of JCML.
Genetic abnormality 95% Philadelphia chromosome Philadelphia chromosome absent
Monosomy chr. No. 7 present in 30%cases.
course Chronic or subacute course going into blast crisis More acute and severe.
Physical findings Splenomegaly most common LAP and hepatomegaly much more than CML
Skin involvement in form of eczema, xanthoma, café  au lait spots is greater.
Blood picture WBC count >1,00,000/mm3
Mild anemia
Thrombocytosis common
LAP score decreased
Fetal Hb normal
WBC count <1,00,000/mm3
 Anaemia more common
Thrombocytopenia +
LAP score normal or decreased
Fetal Hb increased
Monocytosis seen
Treatment Definite T/t: BMT
T.O.C imatinib mesylate


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