Leukemia (Blood Cancer)

Bharat R Agarwal
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AML - Presentation
These children present either with very few symptoms or with severe sepsis, bleeding etc, which indicate marrow failure and involvement of extramedullary organs. Certain features are unique to a particular sub-type of AML for e.g.
- Patients with acute promyelocytic (M3) leukemia may have intravascular coagulopathy due to clotting factor deficiency resulting in severe bleeding.
- Patients with M4 & M5 AML(monocytic component) may have associated gingival hypertrophy from leukemic infiltration, They may also develop meningeal leukemia, retinal infiltration, leukemia cutis or other localized leukemia infiltrations. They may also have chloromas, which are granulocytic sarcomas seen commonly in the skin and have a dull green color due to the high peroxidase content in the leukemia cell.
In addition, in patients with AML, 1/3 of patients may splenomegaly. Hyper-uricemia is also very common.

JMML is sub classified into:
Juvenile chronic myelogenous leukemia (JCML) when there is:
- JMML morpholo1gy in the absence of t(9;22) translocation
- Raised HbF (> 10%)
- No monosomy 7
Infantile monosomy 7 syndrome when there is:
- Presenting age less than 4 years with any type of myelodysplasia
- Monosomy 7

Frequency
The incidence of JMML is 0.6 cases per year per million children. JMML accounts for 18% of all cases of myelodysplastic syndrome in children less than 15 years of age.

Genetics
Seven percent of JMML cases have been associated with neurofibromatosis, type 1. JMML has been described in identical twins.

Age of onset
Forty percent of cases occur before 1 year of age and 60% of cases occur before 2 years of age.

Sex
The male: female ratio is 2.1:1

Clinical symptoms
JMML presents with fever and respiratory symptoms

Physical findings
These include facial rash, lymphadenopathy, splenomegaly and bleeding problems, pharyngotonsillitis and bronchiolitis


Leukemia (Blood Cancer) Leukemia (Blood Cancer) 2/2/2001
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