Chronic Final / Wong: 1003-16
I.Description:
A.Most common childhood cancer.
B.Broad term for a group of malignant diseases of the bone marrow & lymphatic system.
C.Proliferation of immature WBC’s (blast cells) in the blood forming tissues.
D.Blast cells do not respond to body’s feedback system.
II.Etiology
A.Peak age of onset 2 - 6 years
B.10 - 20 times more common in Down Syndrome children
C.Occurs more in caucasian children
D.More common in boys after age 1 year
E.27,000 adults, 2,000 children diagnosed in the U.S. per year
Classification
F.Acute Lymphocytic Leukemia - ALL
G.Acute Myelocytic Leukemia - AML
H.Chronic Lymphocytic Leukemia - CLL
I.Chronic Myelocytic Leukemia - CML
III.Acute Lymphocytic Leukemia
A.Involve the lymphoid or lymphatic system
B.May be B- cell, T - cell, or non-T, non-B
C. 80 % of cases in children
D.20 % of adult cases (higher incidence over 65 yrs)
IV.Acute Myelocytic Leukemia
A.Involve myeloid cells
B.15 - 20 % of cases in children
C.80 % of adult cases
V.Chronic Lymphocytic Leukemia
A.Characterized by early B- cell lymphocytes
B.Usually seen in males over 50
C.Rare in children
VI.Chronic Myelocytic Leukemia
A.Involves leukocytosis & thrombocytosis with an increased production of granulocytes
B.Seen in older adults
C.Blast crisis
D.1 - 5 % of cases in children
VII.Risk Factors
A.Smoking
B.Long-term exposure to benzene, pesticides or herbicides
C.High-dose radiation exposure from a nuclear reactor accident or an atomic bomb
D.Infection with human T-cell leukemia/lymphoma virus (HTLV-1) or Epstein-Barr
E.Chemotherapy with Mustargen, Matulane, Leukeron, VePesid or Cytoxan
F.Hereditary Syndromes - ataxia telangiectasia, Fanconi’s anemia, Wiskott - Aldrich, Down, Bloom’s Kleinfelter’s, or I-Fraumeni Syndrome
G.Myelodysplastic (preleukemic) syndrome
H.Identical twin or first degree relative with leukemia
VIII.Clinical Manifestations
A.Three Main Consequences:
1.BRUISINGfrom decreased platelet production
2.Mild to profound ANEMIAfrom decreased RBCs
3.INFECTIONSfrom the neutropenia.
B.Other signs
Leukemia – November 11 Lecture / Page 1 of 9Chronic Final / Wong: 1003-16
1.Pallor
2.Bone or joint pain
3.Abdominal pain
4.Lymphadenopathy
5.Malaise
6.Fever of unknown origin
7.Hepatoslenomegaly
8.Abnormal WBC (low or high)
9.Thrombocytopenia
10.Petechiae
11.Sweating, esp at night
Leukemia – November 11 Lecture / Page 1 of 9Chronic Final / Wong: 1003-16
IX.Diagnosis
A.History
B.Physical Manifestations
C.CBC - peripheral smear shows immature leukocytes, frequently with low blood counts
D.Bone marrow aspiration & biopsy
E.Lumbar puncture
F.Bone scan or skeletal survey
X.Treatment
Leukemia – November 11 Lecture / Page 1 of 9Chronic Final / Wong: 1003-16
A.Combined chemotherapy
B.Intrathecal chemotherapy
C.Radiation therapy
D.Bone marrow transplant
E.Stem cell transplant
F.Biological therapy
G.Supportive care
Leukemia – November 11 Lecture / Page 1 of 9Chronic Final / Wong: 1003-16
H.Chemotherapy
1.Induction therapy: achieves a complete remission or less than 5% leukemic cells in the bone marrow. Lasts 4-6 wks. Supportive therapy due to myelosuppression is imperative to protect client from infection and spontaneous hemorrhage.
2.CNS prophylactic therapy which prevents leukemic cells from invading the CNS
3.Intensification therapy which eradicates residual leukemia cells, followed by delayed intensification, which prevents emergence of resistant leukemic clones
4.Maintenance therapywhich serves to maintain the remission phase.
5.Reinduction following relapse
XI.ALL Management–
A.Children
1.Induction Chemotherapy: lasts 4-6 wks
a.Vincristine, L-asparaginase, prednisone/dexamethasone
b.CNS prophylactic treatment reserved for patients who are high risk and those with CNS disease
1.High risk pts. add duanorubicin
2.Methotrexate intrathecally
2.Consolidation/Intensification
a.Intermediate to high dose methotrexate + combo similar to induction
3.Maintenance
a.Daily oral mercaptopurine + weekly methotrexate
b.May give pulses of vincristine & prednisone/dexamethasone
B.Adult
1.Remission Induction
a.Prednisone, vincristine, duanorubicin
2.CNS Prophylaxis
a.Cranial irradiation
b.Intrathecal methotrexate
c.High-dose systemic & intrathecal methotrexate
d.Intrathecal chemo alone
3.Maintenance
a.Cyclophosphamide
b.BMT
XII.AML Management
A.Children
1.Induction
a.Cytarabine, Duanorubicin
b.Intrathecal Chemo
2.Post-remission
a.High-dose Cytarabine
b.Etoposide
c.Daunorubicin
3.Maintenance
a.Used but no proof that it significantly improves long-term cure
B.Adult
1.Induction
a.Daunorubicin, cytarabine
b.Daunorubicin, cytarabine, thioguanine
c.Cytarabine, idarubicin
d.Mitoxantrone, etoposide
C.Post-remission
1.Short-term, intensive cytarabine-based consolidation chemo with more doses
2.Intensive cytarabine-based treatment
3.High-dose chemo or chemoradiotherapy with autologous BMT
4.High-dose marrow ablative therapy with allogeneic BMT
XIII.CLL Management
A.Periodic observation
B.Steroids, alkylating agents, purine analogues
C.Combination Chemo
D.Monoclonal antibodies
E.Transplant
XIV.CML Management
A.Chronic phase & Accelerated phase
1.High-dose chemo + BMT
2.Interferon alfa
3.Hydroxyuria
4.Busulfan
B.Blastic phase
1.Vincristine, prednisone, maybe daunorubicin
2.Allogeneic BMT
3.Hydroxyuria (palliative)
4.Radiation therapy
5.High-dose cytarabine
XV.Chemotherapy Side Effects
A.General
1.Immunosuppression
2.Anemia
3.Nausea/Vomiting: Ondansetron in d.o.c. Best to administer before infusion. Best if child never experiences N&V with administrations so conditioned response is not developed.
4.Hair loss
5.Mucositis: greatly compound anorexia because eating is uncomfortable.
6.Thrombocytopenia
7.Anorexia:
8.Fatigue
XVI.Radiation Therapy
A.Site specific
B.Total body irradiation
C.Use in children in low doses or if chemo fails because radiation to brain can cause learning & coordination problems
XVII.Late Effects of Therapy
A.Secondary malignancy – most devastating late effect
B.Treatment with anthracycline associated with cardiomyopathy
C.Cranial radiation & intrathecal chemotherapy are associated with cognitive & neuropsychologic deficits
XVIII.Nursing Considerations
A.Child will receive appropriate primary health care.
B.Child & family will be prepared for diagnostic & therapeutic procedures.
C.Child will experience minimal complications of myelosuppression.
1.Infection
a.Child is most susceptible to infection when:
1.At the time of diagnosis and relapse when the leukemic process has replaced normal leukocytes.
2.During immunosuppressive therapy
3.After prolonged AB therapy that predisposes to the growth of resistant organisms
b.First defense against infection is prevention.
c.Because the usual viral infections of childhood are particularly dangerous, the child is not immunized against these diseases (measles, rubella, mumps, and polio) with live attenuated vaccines until the immune system is capable of responding appropriately to the vaccine. However, may give inactivated vaccines to prevent specific infections (Salk polio, influenza, varicella).
d.TX: GCSF has reduced the incidence and duration of infection in children receiving treatments.
e.Good nutrition helps prevent infection.
2.Hemorrhage:
a.Skin punctures are avoided whenever possible.
b.Provide emotional support.
3.Anemia
D.Problems of irradiation & drug toxicity will be managed.
1.N&V: Ondansetron in d.o.c. Best to administer before infusion. Best if child never experiences N&V with administrations so conditioned response is not developed.
2.Anorexia: assess any factors contributing to the issue.
3.Mucosal irritation: Provide a bland, moist, soft appropriate diet. Use a soft sponge toothbrush. Provide frequent mouthwashes with either saline or sodium bicarbonate (1 tsp baking soda in 1 qt water). Use local anesthetics. DO NOT USE lemon glycerine swabs, hydrogen peroxide, or M.O.M. Avoid viscous lidocaine for young children.
4.Neuropathy: administer stool softeners or laxatives, maintain good body alignment, carry out safety measures during ambulation, provide a soft or liquid diet for severe jaw pain.
5.Hemorrhagic cystitis: increase fluid intake, provide opportunity for frequent voiding, administer drug early in the day to allow for sufficient oral intake and voiding, administer mensa as ordered.
6.Alopecia: warn children, provide soft cotton cap. Teach that hair regrows in 3-6 months.
7.Moon face: assure children that after cessation of the drug the facial changes will return to normal.
8.Mood changes: should warn the parents and encourage them to discuss them with each other and the child. .
E.Child & family will receive adequate support & education
F.Administration
1.Gloves
2.Gown with knit cuff
3.Goggles
4.Prepare in special area
5.Dispose of waste in bag marked for chemo & incinerate
6.Free flowing IV line. Infusion is stopped IMMEDIATELY if any sign of infiltration (pain, stinging, swelling, or redness at the cannulation site) occurs.
7.Anaphylaxis: prevention begins with a careful history for known allergy.
a.Observe for 20 minutes for cyanosis, hypotension, wheezing, severe urticaria.
b.If reaction is suspected:
1.The drug is discontinued,
2.The IV line is flushed with saline,
3.The child’s V/S and subsequent responses are monitored.
8.Premedicate
9.Monitor vital signs
10.Strict I & O
11.Monitor for extravasation
12.Monitor for side effects
G.Pain management
H.Support
XIX.Prognosis
A.Initial WBC count:
B.Age at diagnosis
C.Type of cell involved
D.Sex
E.Karotype analysis
The children with a normal or low WBC count and analysis and who have non-T, non-B ALL and are CALLA positive have a much better prognosis than those with a high count or other cell types. Children diagnosed between 2 and 9 years of age have consistently demonstrated a better outlook. Girls have a better prognosis than boys. The more rapid the induction of a remission in AML, the better the chance for an ultimate long-term, continuous remission.