This presentation is mainly to focus on approach to Acute Lymphocytic Leukemia. Enjoy

1. Course Outline
 1. Introduction
 2. Definition of leukemia
 3. Etiology
 4. Epidemiology of childhood acute lymphoblastic leukemia
 5. Clinical presentation
 6. Diagnosis
 7. Management
 8. Complications
 9. Prognosis
 10. Prevention

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

17. Complication cont’
3. Sepsis
4. Bleeding
5. Seizures
6. Encephalopathy
7. Thrombosis
8. Cognitive defects
9. Secondary malignancy

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

20. Prevention
 Prevention is based on prevention of environmental risk factors
Which stated in etiology

21. References:
1. www.uptodate.com
2. www.medscape.com
3. Acute lymphocytic leukemia article by Yana Puckett, Onyee Chan

22. Thank
Your inputs are welcome