2. General Objective
At the end of the session, participants will be able
to explain about leukemias.
2/13/2023 2
3. Specific objectives
At the end of the session, participants will be able
to:
- Explain hematopoiesis.
- Define leukemia.
- Explain the epidemiology of leukemia.
- List the factors predisposing leukemia
- Classify leukemia.
- List the causes of leukemia.
2/13/2023 3
4. Specific objectives
- Enlist the clinical manifestations of leukemia.
- State the diagnostic criteria of leukemia.
- List the differential diagnosis of leukemia.
- Explain the management of leukemia.
- State the prognosis of leukemia.
2/13/2023 4
6. 6
Blast cell
⢠Blast cells are immature precursors of either
lymphocytes (lymphoblasts), or granulocytes
(myeloblasts).
⢠They do not normally appear in peripheral blood.
they can be recognized by their large size, and
primitive nuclei .
⢠Presence of blast cells in blood, signify
ACUTE LEUKEMIA.
2/13/2023
8. 8
Definition
⢠Leuka = white, emia = blood
⢠Leukemia is a type of cancer of blood or
bone marrow.
⢠Characterized by an abnormal increase of
immature white blood cells called "blasts".
⢠Leukemias are the most common cancers
affecting children.
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9. Leukemia in Children
ď§ Acute lymphoblastic leukemia (ALL)
accounts for 73%,
ď§ Acute myeloid leukemia (AML) accounts
for approximately 18%.
ď§ Chronic myeloid leukemia (CML) is rarely
seen, accounting for less than 4%.
10. 10
Epidemiology
â
â
Most common childhood cancer
â 3,000 new cases each year
Demographics:
⢠Males more commonly than females
⢠Whites more than blacks.
⢠More commonly in patients with Downâs
Syndrome.
2/13/2023
17. 17
⢠Epidemiology of ALL
ďź peak incidence in 2 to 6 years
ďź more in boys
ďź median age in adults-35years.
⢠Etiology
ďź environmental and genetic factors
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38. 38
Induction phase 1
Cycle chemotherapy Dose and schedule
Induction Prednisolon or 1mg/kg p.o days 1-28 days
vincristine 1.5mg/m2 i.v weekly once
x 4 weeks
doxorubicin 30mg/m2 i.v weekly once
x 4 weeks
L-Asparginase 1,00,000 u/m2(total dose) in
divided doses of 10,000 u
daily for 10 days
CNS Proph. Methotrexate 12mg IT days 1,8,15,22
2/13/2023
39. CNS prophylaxis
⢠Given the concern of long-term neurotoxicity
and risk of brain tumors following standard
cranial irradiation, experts recommend lower
dose irradiation combined with intrathecal
administration of methotrexate.
2/13/2023 39
40. CNSProphylaxis
ďŹ CNS involvement at diagnosis <5%
â Without prophylaxis, over 80% of patients in CR
will relapse in the CNS
â With prophylaxis, less than 5% have CNS relapse
ďŹ Intrathecal chemotherapy is now the
mainstay
â Sample intermediate risk regimen: IT MTX
alone or âtriple therapyâ: IT cytarabine Day 1 of
CR followed by MTX/hydrocortisone/cytarabine
4/28/2014
2/13/2023 40
41. 41
Reassess
⢠After 4 weeks of phase 1 induction
assess marrow for remission.
⢠If there is remission taper prednisolone and
after 1 week, start phase 2 induction,
⢠If there is no remission give 2 more weekly
doses of vincristine and doxo and then assess,
if still no remission go for alternate regimen.
2/13/2023
42. 42
Induction phase 2
Inductio
n2
Drugs Dose and
schedule
Cyclophosphamide
Cytosine
arabinoside
650mg/m2 i.v days 1
and 15
75mg/m2 i.v x 4 days a
weeks for 4 week
Methotrexate 12mg/m2 IT days
1,8,15,22
Cranial radiation 200 cGy x 9days
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44. Maintenance phase
duration- upto 2 years
maintena
nce
drug Dose and schedule
1st
month
methotrexate 12.5mg i.t on day 1
vincristine 1.4mg/m2 .v day 1
prednisolone 1mg/kg p.o daily day 1-7
6 mercaptopurine 60mg/m2 p.o. daily for
next 3 weeks
methotrexate 15mg/m2 p.o. once a
week for 3 weeks.
2nd
month
4/28/2014
6 MCP and
T.Methotxerate for
4 weeks. 42
2/13/2023 44
45. 45
Follow up
⢠If the patient completes chemotherapy for 2
years without relapse-stop chemo and follow
up.
⢠No relapse within 5 years -can be declared as
cured.
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46. 46
Allogenic stem cell transpantation
⢠Usually done in second remission.
⢠Can be done in first remission in high risk
patients
ďą WBC>25000,
ďą philadelphia chromosome positive,
poor initial response to remission
induction.
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48. SUPPORTIVE CARE
⢠use of packed red cells
⢠When high fever and possible septicemia
occur in the presence of neutropenia,
antibiotic therapy should be started.
(NEUTROPENIA REGIME)
⢠Platelet transfusions should be
administered to patients with overt bleeding
or when the platelet count is below
10,000/mm3.
49. Nursing management
Assessment
- General condition, activity, alertness
- Pain, fever
- Bleeding
- Intake of child
- Side effects of chemotherapy
2/13/2023 49
50. Nursing diagnosis
1.Acute Pain related to physical agents, e.g.,
enlarged organs/lymph nodes, bone marrow
packed with leukemic cells.
2.Risk for Deficient Fluid Volume related to
decrease fluid intake and increase loss.
3.Imbalanced nutrition: less than body
requirement related to cachexia.
4.Risk for Infection related to inadequate
secondary defense.
2/13/2023 50
51. Nursing diagnosis
4. Activity Intolerance related to generalized
weakness and increased metabolic rate from
massive production of leukocytes.
5. Deficient Knowledge related to lack of
information.
6. Anxiety related to unfamiliar disease condition.
2/13/2023 51
52. Course of treatment
⢠The average duration of treatment in ALL is
24- 30 months, with no advantage of extending
treatment beyond 3 years.
⢠The outcome of ALL remains poor in infants,
even with intense therapy including stem cell
transplant.
2/13/2023 52
53. Late Effects of Treatment
- cranial irradiation: cognitive and intellectual
impairment and development of CNS neoplasms.
- There is a risk of secondary AML after intensive
use of epipodophyllotoxins (etoposide,
teniposide).
- Endocrine dysfunction leads to short stature,
obesity, precocious puberty, osteoporosis, thyroid
dysfunction and growth hormone deficiency.
- Patients with prior therapy with an anthracycline
are at risk of cardiac toxicity.
2/13/2023 53
54. Prognostic factors in ALL
Determinants Favourable Unfavorable
WBC Counts <10,000 >2,00,000
Age 2-10 years <1yr,>10yr
Gender female male
Ethnicity white black
Node,liver,splenomegaly absent massive
Testicular enlargement absent present
CNS involvement absent Csf blast and pleocytosis
FAB Type L1 L2
Cytogenetics T(12;21)(TEL-AML1)
Trsomies 4,10,17
t(9;22)(bcr-abl)
t(4;11)(MLL-AF4)
Ploidy hyperdipoidy hypodiploidy
T
4/2
i8
m
/20
e
14
to remission <14days >28days 48
2/13/2023 54
55. Acute Myeloid Leukemia
- It happens when the body makes too many
immature white blood cells. These cells,
called myeloid blasts, can't mature into normal
white blood cells.
- Because AML develops and gets worse
quickly, prompt treatment is very important.
- Of kids who have leukemia, 20% have AML.
2/13/2023 55
56. Clinical features
â˘swollen gums
â˘infections (like bronchitis or tonsillitis) that keep
coming back
â˘night sweats
â˘belly pain (caused by the build-up of cells in
organs like the kidneys, liver, and spleen)
2/13/2023 56
57. Management
- Chemotherapy: The treatment goal is remission,
which is when tests don't find any cancer cells in the
body.
- Then, maintenance chemotherapy is used to keep
the child in remission and prevent the cancer from
coming back. The child will get maintenance chemo
for 2 to 3 years.
- Stem cell transplant
2/13/2023 57
58. Chronic Myeloid Leukemia
- It constitute 3% of leukemias in childhood.
- It is characterized by myeloid hyperplasia of
the bone marrow, extra medullary
hematopoiesis, elevation of white blood cell
count.
2/13/2023 58
59. Chronic Myeloid Leukemia
Adult CML: The condition is clinically and
hematologically comparable to the adult form of
chronic myelogenous leukemia and occurs in
children above the age of 4 years.
Juvenile CML: This form presents in infancy and
early childhood, usually below the age of 4 years,
and has a more rapid course.
2/13/2023 59
60. Clinical features
chronic phase
- fatigue,
- malaise,
- weight loss,
- excessive sweating,
- abdominal fullness, and
- bleeding due to platelet dysfunction
- splenomegaly is usually massive.
- Symptoms of leukocytosis such as headache,
dizziness and visual disturbances.
2/13/2023 60
61. Treatment
Aim:
To control increasing white cell counts.
- First and second-generation oral tyrosine
kinase inhibitors (TKI) are the treatment of
choice.
- imatinib therapy.
- The starting dose of imatinib is 340 mg/ m2/
day.
2/13/2023 61
62. Treatment
- Bone marrow cytogenetics: 6 monthly until a
complete cytogenetic response is obtained.
- Allogeneic stem cell transplantation is
recommended for patients who do not respond
to TIU.
2/13/2023 62
63. Chronic Lymphocytic Leukemia
- Chronic lymphocytic leukemia (CLL) is a type
of cancer of the blood and bone marrow.
- Chronic lymphocytic leukemia most commonly
affects older adults.
- There are treatments to help control the disease.
2/13/2023 63
64. Facilities in Nepal
- Bipanna Nagarik Kosh was started after the
Janandolan of 2062 BS.
- Cardiovascular diseases, Cancer, Renal failure,
Alzheimer's disease, Parkinson's disease, Head
and Spinal injury, Sickle Cell Anaemia and
Stroke are covered under this program.
65. Facilities in Nepal
Those eligible to claim services under the Bipanna
Nagarik Kosh will receive the following:
1.Each patient will be provided NRs. 1,00,000/- as
health care expense including medicines required
for disease management
2.For patients with renal impairment (ऎŕĽŕ¤ŕĽŕ¤˛ŕ¤ž रŕĽŕ¤ŕĽŕ¤ŕĽ
ŕ¤ŕ¤Şŕ¤ŕ¤žŕ¤° सऎŕĽŕ¤Źŕ¤¨ŕĽŕ¤§ŕ¤Žŕ¤ž)
3.Free hemodialysis and peritoneal dialysis for 1
year
66. Facilities in Nepal
1.NRs. 2,00,000/- as kidney transplant expense
2.NRs. 1,00,000/- in one or more instalments as
medicine expense for post-transplant management
3.NRs. 1,00,000 to purchase medicine post kidney
transplant is provided as cash grant. All other
subsidies are provided directly through hospitals
in Nepal.
2/13/2023 66
67. Facilities in Nepal in listed hospital
for Cancer
1.National academy of health sciences, Bir
hospital, Kathmandu
2.Tribhuwan University, Teaching Hospital,
Kathmandu
3.Patan Academy of Health Science, Patan
4.B.P. Koirala Institute of Health Science, Dharan
5.Maternity Hospital, Kathmandu
6.Civil Service Hospital, Kathmandu
7.Kanti Children hospital, Kathmandu
68. Facilities in Nepal in listed hospital
for Cancer
1.B.P. Koirala Memorial Cancer Hospital,
Bharatpur
2.Chitwan Medical College Teaching Hospital,
Chitwan
3.Bhaktapur Cancer Hospital, Bhaktapur
4.Cancer Care Nepal, Lalitpur
5.Nepal Cancer Hospital & research centre
6.Kathmandu Cancer Center, Tathali, Bhaktapur
2/13/2023 68
69. REFERENCES
⢠Ghai O, Paul V, Bagga A. Essential Pediatrics. 7th ed. CBS
Publisher & Distributers; 2008.
⢠Before you continue to Google Search [Internet]. Google.com.
2021 [cited 20 April 2021]. Available from:
https://www.google.com/search?q=fab+classification+of+leuke
mia&oq=FAB+classifica&aqs=chrome.0.0l2j69i57j0l7.12372j0j
15&sourceid=chrome&ie=UTF-8
⢠Blinatumomab Effective for Children with Relapsed Leukemia
[Internet]. National Cancer Institute. 2021 [cited 20 April 2021].
Available from: https://www.cancer.gov/news-events/cancer-
currents-blog/2021/blinatumomab-relapsed-b-cell-leukemia-
children-young-adults
⢠S et al. Leukemia [Internet]. WebMD. 2021 [cited 20 April
2021]. Available from:
https://www.webmd.com/cancer/lymphoma/understanding-
leukemia-basics
2/13/2023 69
71. Reinduction
Re
induction
Drug Dose and schedule
Vincristine 1.5 mg/m2 i.v weekly one
dose on day 1 and 8
Doxorubicin 30mg/m2 i.v. weekly one
dose on day 1 and 8
4/28/2014
Prednisolone 1mg/kg p.o daily for 14
days
40
2/13/2023 71
Hinweis der Redaktion
(i.e. the nucleus contain nucleoli).
less studied
Diamond-Blackfan anemia is a disorder that primarily affects the bone marrow.
Neurofibromatosis
Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome.
Li-Fraumeni syndrome (LFS) is an inherited familial predisposition to a wide range of certain, often rare, cancers. This is due to a change (mutation) in a tumor suppressor gene known as TP53.
Bloom syndrome (BSyn) is a rare genetic disorder characterized by short stature; a sun-sensitive, red rash that occurs primarily over the nose and cheeks; mild immune deficiency with increased susceptibility to infections; insulin resistance that resembles type 2 diabetes; and most importantly,
Epipodophyllotoxins are substances naturally occurring in the root of American Mayapple plant (Podophyllum peltatum).Â
ITPâ
isolated thrombocytopenia,
well child with
no lymph node enlargement or spenomegaly
Aplastic Anaemia
Pancytopenia with
no organ enlargement
Juvenile Rheumatoid Arthritis
Infectious mononucleosis
Atypical lymphocytes
Metastatic solid tumours
Allogeneic stem cell transplantation involves transferring the stem cells from a healthy person (the donor) to the patient's body after high-intensity chemotherapy
helps to clear uric acid from the blood.Â
Rasburicase is a medication that helps to clear uric acid from the blood. It is a recombinant version of urate oxidase, an enzyme that metabolizes uric acid to allantoin. Urate oxidase is known to be present in many mammals but does not naturally occur in humans
Leukapheresis (/ËluËkĘfÉËriËsÉŞs/ ( listen)) is a laboratory procedure in which white blood cells are separated from a sample of blood. It is a specific type of apheresis, the more general term for separating out one particular constituent of blood and returning the remainder to the circulation.
The goal of this to eradicated leukemia as such at the end of this phase there are <5% leukemia blasts in the bone marrow.
Most children with leukemia have s subclinical CNS involvement at diagnosis, which might act aa sanctuary where blasts are protected because of the blood-brain
barrier. CNS prophylaxis has enabled increase survival rates in leukemia.
Intrathecal administration is a route of administration for drugs via an injection into the spinal canal, or into the subarachnoid space so that it reaches the cerebrospinal fluid (CSF) and is useful in spinal anesthesia, chemotherapy, or pain management applications.
methotrcxate, hydrocortisone and cytarabinc without cranial irradiation or high dose systemic chemotherapy.
Remission means that the signs and symptoms of your cancer are reduced. Remission can be partial or complete. In a complete remission, all signs and symptoms of cancer have disappeared. If you remain in complete remission for 5 years or more, some doctors may say that you are cured
Use of these medications may result in significant granulocytopenia and need supportive care.
Sometimes, despite the best care and significant progress made in treatment, cancer comes back. When this happens it is called a recurrence or relapse.
after taking appropriate blood cultures and a chest radiograph
Hyperdiploidy (chromosomal number 51â65) is a common cytogenetic abnormality in pediatric patients with Bâlymphoblastic leukemia (BâALL)
(tiny red spots on the skin caused by easy bleeding)
(with appearance¡ of the complete range of granulocyte precursorn in the peripheral blood).
Imatinib is in a class of medications called kinase inhibitors. It works by blocking the action of the abnormal protein that signals cancer cells to multiply. This helps stop the spread of cancer cells.
With the country slowly recovering from the decade long armed conflict, this Kosh was established to provide some financial relief to people from difficult and expensive diseases.