2. Childhood leukemia
• Childhood leukemia is a type of leukemia, usually acute
lymphocytic leukemia (ALL), and a type of childhood cancer. The
cure rate of childhood leukemia is generally higher than adult
leukemia, approaching 90%, although some side effects of
treatment last into adulthood
3. • Leukemia is a hematological malignancy or a cancer of
the blood. It develops in the bone marrow.
• When a child has leukemia, the bone marrow produces white
blood cells that do not mature correctly.
• Normal healthy cells only reproduce when there is enough space
for them.
• The body will regulate the production of cells by sending signals
of when to stop production.
• When a child has leukemia, the cells do not respond to the
signals telling them when to stop and when to produce cells,
regardless of the available space.
4.
5. Types
1. Acute Lymphocytic Leukemia (ALL)
2.Acute Myelogenous Leukemia (AML)
3.Chronic Myelogenous Leukemia (CML)
(CML is rare in children)
6. Acute lymphocytic leukemia
• Acute lymphocytic leukemia (ALL), which accounts for about 3
out of 4 cases of leukemia in children.
• ALL is a form of leukemia that affects the lymphocytes, a type of
white blood cells which fights infection.
• When a patient has ALL, the bone marrow makes too many
immature white blood cells and they do not mature correctly.
Therefore, the white blood cells over-produce, crowding the
other blood cells. The white blood cells also do not work
correctly to fight infection.
8. Bone marrow smear (large
magnification) from a patient with
acute lymphoblastic leukemia
9. Acute Lymphocytic Leukemia
• ALL causes damage and death by crowding out normal cells in
the bone marrow, and by spreading (infiltrating) to other organs.
ALL is most common in childhood with a peak incidence at 2–5
years of age, and another peak in old age.
• Cure is a realistic goal, as ≥94% of children have continuous
disease-free survival for five years and appear cured, while 30–
40% of adults have continuous disease-free survival for five
years.
10. Signs and symptoms
• Generalized weakness and fatigue
• Anemia
• Frequent or unexplained fever and infection
• Weight loss and/or loss of appetite
• Excessive and unexplained bruising
• Bone pain, joint pain (caused by the spread of "blast" cells to the surface of
the bone or into the joint from the marrow cavity)
• Breathlessness
• Enlarged lymph nodes, liver and/or spleen
• Pitting edema (swelling) in the lower limbs and/or abdomen
• Petechiae, which are tiny red spots or lines in the skin due to
low platelet levels
11. Acute Myelogenous Leukemia (AML)
• Acute Myelogenous Leukemia (AML) – A very rapid production of
abnormal white blood cells known as the myelocytes. This form of
Leukemia strikes children as well as adults.
• Accounts for 20% of all pediatric cases
• Chronic cases of leukemia are also present; these are very slow
growing cancers affecting myelocytes and lymphocytes.
• In a chronic case, the white blood cells are more mature and they are
produced at a much slower rate
13. Pathophysiology
• The underlying pathophysiology in AML consists of a maturational arrest
of bone marrow cells in the earliest stages of development.
• It involves the activation of abnormal genes through chromosomal
translocations and other genetic abnormalities.
• The mechanism of this arrest is under study, but in many cases, it
involves the activation of abnormal genes through chromosomal
translocations and other genetic abnormalities.
• Second, the rapid proliferation of these cells, along with a reduction in
their ability to undergo programmed cell death (apoptosis), results in
their accumulation in the bone marrow, the blood, and, frequently, the
spleen and liver.
14. Signs and symptoms
Pediatric acute myelocytic leukemia (AML) can be divided into the following:
(1) those caused by a deficiency of normally functioning cells
(2)those due to the proliferation and infiltration of the abnormal leukemic
cell population
(3) constitutional symptoms.
15. Symptoms due to a deficiency of normally functioning cells
include the following:
• Cytopenias: Can result from a deficiency of normally functioning cells
• Anemia: Characterized by pallor, fatigue, tachycardia, and headache
• Hemorrhage: Most commonly, easy bruising, petechiae, epistaxis,
gingival bleeding
• Fever: Should initially always be attributed to infection
16. Symptoms due to the proliferation and infiltration of
the abnormal leukemic cell mass and infiltrative
disease include the following:
• Extramedullary infiltration: Most commonly in the reticuloendothelial
system
• Mediastinal mass: May cause symptoms of respiratory insufficiency or
superior vena cava syndrome
• Abdominal masses: May cause pain or obstruct the GI or urogenital
tracts
• Gingival hyperplasia, CNS infiltration: Often associated with
monoblastic leukemia
17. Constitutional and miscellaneous symptoms
• Unexplained, persistent fevers
• Weight loss
• CNS symptoms, although uncommon initially, can appear during
follow-up with various findings.
• In rare cases, leukemic cells infiltrate all parts of the eye. The retina
and iris are the sites most commonly affected. Iritis often causes
photophobia, pain, and increased lacrimation, whereas retinal
involvement is often accompanied by hemorrhage and can lead to a
loss of vision.
18. Testing
• Blood counts with differential: WBC counts may be decreased or elevated;
platelet counts usually low
• Blood smears: Primitive granulocyte/monocyte precursors observed; Auer
rods present in specimens of circulating blood from many AML patients
• Blood chemistries: Frequently elevated serum uric acid, serum
muramidase (lysozyme), LDH levels
• Blood and urine cultures: Always obtain in a child with fever and leukemia
• Coagulation tests: Perform with initial diagnosis for evidence
• Histochemical staining: Standard Wright-Giemsa stains and histochemical
stains to differentiate the various acute leukemias.
19. Conti..
• Immunophenotyping: To further characterize leukemic cells for
different cell lineages and stages of development.
• Cytogenetic testing: To confirm the diagnosis and for prognostic
purposes
20. Imaging studies
• Imaging studies are not required for the diagnosis of AML in children,
but the following radiologic studies can be helpful in managing
complications that arise:
• Radiography: Routine CXR to rule out mediastinal masses; abdominal
images in patients with abdominal pain and distention to rule out
perforation; extremity images in patients to rule out metaphyseal
bands at the distal femurs (mostly in pediatric ALL), periosteal new
bone formation, focal lytic lesions, or pathologic fractures.
21. Conti…
• MRI or CT scanning of the head, spine, or other affected areas: For
patients with neurologic symptoms to rule out intracranial hemorrhage
or infiltrative disease
• CT scanning of abdomen or sinuses: For abdominal pain or
suspected infection of the large bowel; for early detection of
asymptomatic sinusitis as cause of persistent, unexplained fevers
• Echocardiography: To exclude serious infections that affect heart
function; also, perform before chemotherapy and periodically with
administration of high cumulative doses of chemo drugs.
22. HLA Typing
• Human leukocyte antigen (HLA)–matched family donors should be
identified because bone marrow transplantation (or hematopoietic
stem cell transplantation) may be considered in high-risk patients.
• At the time of diagnosis, the donor screening process should be
started by obtaining blood for HLA matching from the patient and
immediate family members.
23. Procedures
• Bone marrow examination: To establish the diagnosis of AML
• Lumbar puncture and CSF examination: For diagnostic and
therapeutic purposes
24. Management
Two goals
(1) destroying the leukemic cells as rapidly as possible and preventing
the emergence.
(2) supporting the patient through long periods of pancytopenia until
their bone marrow achieves hematologic remission and is again
producing normal hematopoietic cells.
26. Nonpharmacologic therapy
AML may also be managed with nonpharmacologic treatments such as
the following:
• Allogeneic or autologous BMT following chemotherapy and irradiation:
May reduce relapse rates but doesn’t always improve overall survival
• Radiation treatment: Primarily to treat chloromas and other masses
pressing on a vital structure and that may imminently cause
irreversible damage; craniospinal irradiation for persistent CNS
leukemia
• Transfusion support: To correct anemia and thrombocytopenia until
remission is achieved (eg, RBC transfusions); to correct
coagulopathies (FFP)
27. Surgical options
The role of surgery in AML is limited and may
include the following:
• Placement of a central venous catheter: To begin
treatment and to manage all aspects of
chemotherapy and transfusion support
• Biopsy or aspiration of tissue for culture: To detect
possible abscess in febrile patients
28. Classification
• The World Health Organization (WHO) has classified acute myeloid
leukemias into groups, although this classification is rarely used in
pediatrics. However, for general purposes, note the following:
• Acute myeloid leukemia with characteristic cytogenetic translocations
(eg, promyelocytic leukemia with typical t[15;17])
• Acute myeloid leukemia with multilineage dysplasia
• Acute myeloid leukemia and myelodysplasia syndromes secondary to
therapy (eg, those following alkylating agents)
• Acute myeloid leukemia not otherwise categorized (eg, erythroid
leukemias, monocytic leukemias)
29. Prognosis
• With an overall survival rate of 45-60%, the prognosis for children
with acute myeloid leukemia has improved significantly since the
late 20th century.
• A Japanese consortium reported an overall 5-year survival rate of
62%.The long-term, disease-free survival rate is approximately
65% for patients receiving human leukocyte antigen (HLA)–
matched stem cell transplants from family donors, but, as with
chemotherapy, this rate is lower in high-risk patients. When
patients die during treatment or after relapse, the cause is most
commonly infection, bleeding, or refractory disease.
