1. Neoplastic disorder

2. Neoplastic disorder
Neoplastic disorder: are the leading cause of death from disease in children.
Children cancer involve blood and blood forming organ and solid tumor.

3. leukemia Leukemia: is the cancer of the blood-forming tissues.
It is the most common form of childhood cancer.
It occur more frequently in males than in females after age 1 year.
Peak onset between 2-6 years.
3-4/ cases less than 15 years annual.
It is not an inherited disease but there may be genetic link.
Down syndrome and certain type of blood disorder also increase the risk of developed leukemia.

4. leukemia Morphology classification:
1. Acute lymphoid leukemia (ALL), lymphoblastic, lymmphoblastoid, lymphatic, lymphocytic. Stem cell, blast cell , common type in younger child.
2. Acute non lymphoid (myelogenous) leukemia ANLL or AML. granulocytic, myelocytic, monocyte, myelogenous,monoblastic, monomyeloblastic, occur in both adult & child.
3. Chronic lymphoid leukemia. CLL in adult never affect child.
4. Chronic myeloid leukemia. CML in adult some times in children.

5. leukemia Causes: leukemia are due to mutation in the DNA:
Chromosomal translocation.
Crossing over of parts of chromosomes to other.
Disrupting specific genes that mediate cell divisions rate mutation may occur spontaneously or as a result exposure to :
Radiation
Carcinogenic substances.
Viruses__ T-cell leukemia may be virus cause.
Electromagnetic fields example of carcinogic.

6. leukemia
Pathophysiology: leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissue of the body,
so the resulting pathologic condition and clinical manifestation are caused by:
infiltration and replacement of any tissue of the body with non-functional leukemic cells.
Spleen & liver are the most severely affected see table 26-2

7. leukemia Diagnostic evaluation: History and physical manifestation.
peripheral blood smear: immature form of leukocytes.
Bone marrow aspiration: flow cytometry to identifies the specific type of blast cell and bone marrow hypercellular.
LP: to determine if there is any CNS involvement.
Several chemical stains aid in differentiation between ALL & AML :
Cytochemical markers.
Chromosomal studies.
Cell-surface immunologic markers. cell surface antigens differentiation of ALL into three blood classes:
Non -T. non B- ALL__ best prognosis if have common ALL antigen CALLA positive.
B-cell ALL.
T-cell ALL.

8. leukemia
Therapeutic management: treatment of leukemia involves the use of chemotherapy, with or without cranial irradiation, and some cases bone marrow transplant.
Four steps of treatment:
1. Remission induction: a complete remission or less than 5% leukemic cell in the bone marrow .
Almost immediately after confirmation of the diagnosis.
Last for 4-6 weeks.
ALL drug used: corticosteroid, Vincristine, L-Asparagines with or without doxorubicin.
For AML Doxorubiein, Cytosare, Etopside or Thiagnanine.
The period after a remission can be critical.

9. leukemia
2. CNS prophylactic therapy: prevent leukemia cells from invading the CNS by: using chemotherapy intrathecally ( methotrexate, cytarapin and hydrocortisone) BBB.
3. Intensification therapy (consolidation): eradicates residual leukemic cell, followed by delayed intensification to prevent emergence of resistant leukemic clones.
Intrathecally (IT), systemic chemotherapy including drug L-asparagines, high dose or intermittent MTX,CYTARABIN, VINCRISTINE AND 6-MP.
These treatment for several month.
4. Maintenance therapy: serve to maintain the remission phase, begin after completion of successful induction remission and consolidation therapy to prevent the remission and further lessen the number of leukemic cell.
Drug: daily mercaptopurine, weekly MTX and periodic IT therapy.

10. leukemia Remaining 2 years period.
CBCs are taken to evaluate the marrow's response to drug.
Re-induction after relapse:
Relapse: the presence of leukemic cell in the bone marrow, CNS or tests once again.
Therapy for the child include re-induction with Predniselone and previously used.
Prognosis: the most important prognostic factor for determining long term survival for children with ALL, in addition to treatment:
The initial WBCs count . Low or normal count have much better prognosis.
The child's age: at the time of diagnosis.2-9 years better than below2 or after 10 .

11. leukemia
The type of cell involved : non-T, non-B ALL & CALLA positive are better prognosis than T-cell ALL or B-cell ALL.
Late effect of treatment:
Almost every anti-Neoplastic agents including chemotherapy and irradiation cell cause:
Secondary malignant e.g. cranial irradiation at age 5 years or younger__ brain tumor.
Treated with anthracycline__ cardiomyopathy.
Cranial irradiation and IT chemotherapy__ cognitive and neuropsychological deficits.