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Childhood Leukemia��Dr. SHEHU M

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Outline

Introduction
Etiology
Classification
Clinical features
Investigation
Treatment
Prognosis
Conclusion

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Introduction

A group of malignant disorders affecting the blood and blood-forming tissues of

Bone marrow
Lymph system
Spleen

Occurs in all age groups

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Introduction

Most frequent neoplasm in children (41%)
45/ 1million children under the age of 16 years
Incidence peak at 2 – 5 years
75-80% -ALL
15-20% - AML <5% - CML

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Etiology �

Unknown
Higher risk in congenital disorders: -trisomy 21 (14 times higher) -Turner syndrome -Klinefelter syndrome -neurofibromatosis type 1 -Fanconi anemia

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Etiology

-Diamond - Blackfan syndrome -Turner syndrome -Klinefelter syndrome -Bloom syndrome

-Ataxia- teleangiectasia -Severe combined immuno deficiency -Twining

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Etiology

Associated with the development of leukemia

Chemical agents
Chemotherapeutic agents
Viruses
Radiation

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Classification�

Acute versus chronic

Cell maturity

Acute: clonal proliferation of immature hematopoietic cells (the formation of blood or blood cells )
Chronic: mature forms of WBC; onset is more gradual

Nature of disease onset

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Classification

Type of white blood cell (WBC)

Acute lymphocytic leukemia (ALL)
Acute myelogenous leukemia (AML)

Also called acute nonlymphoblastic leukemia (ANLL)

Chronic myelogenous leukemia (CML)
Chronic lymphocytic leukemia (CLL)

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ALL

80% of leukemias
Girl – to- boy ratio is 1: 1.2
Peak incidence 2 – 5 years
Incidence in white children is twice as high as in nonwhite children

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Clinical features/ History

Fatigue
Pallor
Anorexia
Bruising/Bleeding
Fever
Bone/joint pain
CNS symptoms

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Examination findings

Pallor
Bruises
Petechiae
Lymphadenopathy
Hepatosplenomegaly
Cranial Nerve Palsies
Testicular enlargement
Chloromas

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Diagnosis/ differentials

History and P.E
Other malignancies
ITP
Rheumatoid arthritis
Aplastic anaemia
Leukemic reaction from infections

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Investigation

Red cells: -hemoglobin – low, low retics
White blood cell : - normal/ low/ high -in children with high WBC- leukemic blast cells present
Platelets: -usually low

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Investigation

Bone marrow analysis: >25% blasts -characterize the blast cells -determine the degree of reduction of normal hematopoiesis -morphological, ALL - L1, L2, L3

-immunological

-biochemical

-cytogenetic analyses

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Investigation

Serum biochemistry: - Serum urea - Serum potassium

- Serum hypocalcemia

- Serum creatinine

- Serum uric acid, LDH

- Serum phosphate

- LFT

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CXY
Coagulation studies
Urine MCS�Stool MCS
Blood MCS
LP for CSF analysis
Abdomino-pelvic USS

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Treatment

Induction (4-6 weeks)
Consolidation (4-8 weeks)
Maintenance

2 years for females
3 years for males

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Treatment

Combination chemotherapy
Vincristine
Steriods
Methotrexate
Cytarabine
L- Asparaginase
6-Mercaptopurine

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Treatment

Antibiotics
Antifungals
Trimethoprim- Sulfamethoxazle
Alkalization of urine
Oral Allopurinol
Proguanil
Hydration

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Blood transfusion
Platelets concentrates
Anti-emetics
High calorie diets
G-CSF
Nursing care
Bone marrow Transplant

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Complications from treatment

Tumor Lysis Syndrome
Infection/Sepsis
Hemorrhage / DIC
Leukostasis
Cardiotoxicity
Secondary malignancies
Endocrine abnormalities

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Prognosis

Favorable:

WBC <10x10 ⁹/l
Age 2-7
Female
Response on treatment
Pre-B-ALL
Hyperploid

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Prognosis

Favourable

FAB L1
LDH- moderate
White race
No CNS involvement
No organomegaly
No mediasternal mass

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Prognosis

Unfavorable:

WBC >50 x 10 ⁹/L
Age < 2 and >10
Male
Response on treatment (-)
Hypoploid, t(9;22)/t(9;11)
FAB L2/L3

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Prognosis

Unfavorable:

↑↑LDH high
Visceromegaly
Mediasternal mass
Black race
CNS involvement
Mature B or T cells

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Risk Stratification

Low Risk
Average (Standard) Risk
High Risk
Very High Risk

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AML

Proliferation of myeloid tissue
Abnormal increase

-granulocytes

-myelocytes

-myeloblasts in the circulating blood

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AML

15-20% of all leukemias in children
Remains stable throughout childhood
increase during adolescence
No difference in incidence between boys and girls

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FAB classification

M0: immature myeloblastic leukemia
M1: myeloblastic leukemia
M2: myeloblastic leukemia + maturation
M3: promyelocytic leukemia
M4: myelomonocytic leukemia
M5: monocytic leukemia
M6: erythroleukemia
M7: megakaryocytic leukemia

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Conclusion

Early diagnosis and treatment
5 years survival rate of >80% for ALL
5 years survival rate of < 65% for AML

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AML

Clinical features
Similar to ALL

- > CNS symptoms, subcut. Nodules, DIC

Diagnosis - > 30% of myeloid cells in BM
Treatment

- Induction phase 1 & 11

- Intensive phase

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Questions?

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BEST WISHES

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