2. Introduction
Leukemia is a type of cancer of blood or bone marrow characterized by proliferation of
immature white cells,
Acute lymphoblastic leukemia (ALL) is a malignancy of B or T lymphoblasts characterized
by uncontrolled proliferation of abnormal, immature lymphocytes and their progenitors
which ultimately leads to the replacement of bone marrow elements and other lymphoid
organs resulting in a typical disease pattern characteristic of Acute Lymphocytic Leukemia
is the commonest pediatric malignant.
ALL is fourfold compared to AML, and account 80 % of childhood.
It occurs in male than in female and more common in white than in black.
Clinical manifestation is related to ; anemia, thrombocytopenia, neutropenia and CNC
manifestation.
3. Epidemiology
Most common malignant of childhood.
4000 people/year of patient under 18 year is diagnosed in USA.
It occurs more in children of Trisomy 21, neurofipromatosis 1, ataxia telangiectasia.
Peak age :2-10 years, but more common between 2-3 years .
Poor prognosis in children under 1 year and adult people.
It’s survival rate is increasing greater than 85% in last five years.
4. Etiology
Etiology of Acute Lymphoblastic Leukemia is unknown.
Some suggest environmental factors such as: exposure to benzene, ionizing radiation
and exposure to previous radiotherapy or chemotherapy.
It is not familial related.
Certain genomic particularly p53.
5. Clinical presentation
ALL clinical presentation mainly related with diminished in blood components.
In history of patient:
Anemia: weakness, dizziness, musculoskeletal pain.
Neutropenia: fever occurred due to infection
Thrombocytopenia: easily bleeding ( epistaxis, gingival bleeding etc..)
Mainly has B-symptoms ( constitutional symptoms) : fever, night sweat, weight loss.
6. Clinical presentation
On physical examination:
Hepatomegaly and splenomegaly( 64%, 61%)
Easily bruising
Joint tenderness
Central or peripheral pallor
Lymphadenopathy which may be identified in history or on P/E
7. Cont’
Some symptoms implicated to involvement of CNS such as headache, lethargy,
nuchal rigidity, vomiting
Testicular enlargement with up to 10 % is due to relapsed leukemia
Mediastinal mass which can cause Superior Vena Cava Syndrome (dysphagia,
dyspnea, or swelling of the neck, face, and upper limbs )
8. Diagnosis
ALL is diagnosed based on clinical presentation and laboratories, imaging modalities, if
you suspect leukemia you have to transfer to pediatric oncology for evaluation,
diagnosis and management.
Testing for: Complete morphologic, Immunophenotypic characterization, genetic and
leukemic cells
Laboratories: CBC, electrolytes and renal panel, LDH level.
If patient has respiratory symptoms do chest x-ray
9. Diagnosis cont’
If has abdominal tenderness do CT-scan of abdomen and pelvis.
Lumbar puncture for CSF ( presence of lymphoblasts) analysis if CNS involvement.
Do bone marrow, lymph node aspiration and biopsy for histopathology study.
Cytochemistry ( Periodic Acid Schiff, Nonspecific Esterase, Myeloperoxidase) for
classification
10. Management of ALL
Before treatment of ALL, you should evaluate:
Laboratories such as :- Chemistry ( Electrolytes, liver and renal function test, LDH, uric acid ,
calcium and phosphate)
- Coagulation profiles ( Prothrombin time, partial prothrombin time, fibrinogen)
Lumbar puncture/Neurological evaluation: before ALL treatment you do lumbar puncture for
evaluation of potential leukemic involvement of CNS inorder to give prophylactic first dose of intrathecal
therapy.
1. CNS-1: no lymphoblasts in CSF regardless of WBC
2. CNS-2: <5 WBC/micro in CSF with presence of lymphoblasts
3. CNS-3: > or = 5 WBC/micro in CSF with presence of lymphoblasts
11. Management cont’
Other pretreatment evaluation includes:
Screen for infection and initiate empirical treatment appropriately
Cardiac evaluation with Echocardiogram ( anticipation of Anthracycline)
Human leucocyte Antigen (HLA) typing during hematopoietic stem cell transplant.
CT scan of chest to evaluate mediastinal mass
12. Management cont’
Management of child with leukemia as for every cancer is multidisciplinary.
You have to transfer to pediatric cancer specialist or center for better follow up and
management.
Treatment of ALL has Three phases:
o Remission-Induction phase ,therapy with mainly corticosteroids, anthracycrine,
vincristine and 1- asparginase
o Intensification/Consolidation therapy widely used and include variety of
chemotherapeutic drugs with good outcomes( cyclophosphamide cytarabine,
dexamethasone, MTX etc…).
13. Management cont’
o Maintenance therapy uses oral 6-mercaptopurine or methotrexate once a week or once a
month.
Additional the drugs directed to CNS consists of systemic chemotherapy that enters the
CSF as well as intrathecal MTX for entire course of treatment
14. Management cont’
The treatment last in between 2-3 years to be completed.
Patient may undergo splenectomy for boosting platelets count but does not affect the
outcome, also radiotherapy is used for reduce splenomegaly.
Patient can be undergo transfusion of all required blood products.
In cancer center they sometime use medication related to type of ALL.
You treat infection accordingly based on culture result.
As for those children has malnutrition, require nutritional support.
15. Complications
Some complications are related to the treatments others due to ALL
1. Tumor lysis syndrome the life threatening complication related to chemotherapy
and characterized with:
Hyperuricemia
Hyperkalemia
Hyperphosphatemia and
Hypocalcaemia
2. Renal failure which is invariable
16. Complication cont’
Luekostasis which is oncologic emergency also TLS,
It may manifest due to stasis of leukocyte in different organs:
1. Respiratory: dyspnea, hypoxemia, respiratory failure
2. Nuerologic involvement : somnolence, coma or focal neurologic deficit
18. Prognosis
The likelihood of long-term cure in ALL depends on the clinical and laboratory features
and the treatment.
o Prognostic risk assessment :
1. Age
2. WBC count
3. Complete remission within 4 weeks
4. Cytogenetics
19. Prognosis cont’
Classification of prognosis based on criteria :
1. Low risk
2. Average or standard risk: age of 1-9.9 years, WBC count less than 50,000 at
presentation, good response to initial treatment, lack of cytogenetic features
3. High risk
4. Very high risk