Katie Davis, Michelle Eads, Kate Jamis, Joe Kussman, Michelle Robbins, TC Roope, & Becky Seifert ALL: Acute Lymphocytic Leukemia Pathophysiolgy Hematopoietic: blood forming, refers to normal cell types usually Lymphoblast: precursor to t-cells, b-cells and NK cells. They differentiate in the bone marrow. B-cell: ingest antigens and manufacture antibodies, these are the ones that go haywire in the most common type of ALL T-cell: called t-cells since they migrate from bone marrow to the thymus where they mature. ALL is the result of clonal proliferation and overabundance of leukemic lymphoblasts in the bone marrow and other lymphoid tissues. May be present in the bone marrow, thymus, liver, spleen, lymph nodes, testes, and CNS. The deviation in B-cell ALL occurs when the cell synthesizes immunoglobulins. Examination of cell differentiation during remission shows differentiated hematopoiesis was restored In others, the remission marrow had the same clonal product as found at diagnosis. Detection of clonal hematopoiesis in remission was associated with relapse in 89% of these cases Like most cancers ALL is caused by genetic damage that can allow 2 things to happen: Faulty expression of the genes that regulate normal development of B cells and T cells (allows the undifferentiated blast cells to become malignant). Corruption of genes that control mutations (anti-oncogenes). Signs & Symptoms Rapid fatigue, Malaise, SOB during activity, Pale complexion, Frequent bruising, Prolonged bleeding, Petechiae, Bone and joint pain, Fever, Enlarged lymph nodes, Headache, Vomiting Incidence: See graph: ALL age-specific incidence rates 2000-2003 Differential Diagnosis Acute Lymphocyctic Leukemia (ALL) Rapid Onset of Symptoms Predomina Lymphoid Cell Line nt Affected Cell Line 15% Incidence Typical Age Symptoms Survival Rates Mostly children (3-4 years old) & adults under the age of 40 Nonspecific fatigue & weakness, bruising, fever, persistent infection, weight loss, abdominal fullness, headaches, nausea, vomiting, & blurred vision Mean survival 5 yrs. with treatment Long term survival 40% in adults 70% for children Complete remission 95% of children Acute Myelocytic Leukemia (AML) Rapid Chronic Lymphocytic Leukemia (CLL) Late Chronic Myelocytic Leukemia (CML) Late Myeloid (granulocytes) Cell Line Lymphoid Cell Line Myeloid (granulocytes) Cell Line 33% 25% 10 to 15% Adults Median age=20 Older Median Age=60 40-60 years old Median age=49 Nonspecific fatigue & weakness, bruising, fever, persistent infection, weight loss, abdominal “fullness”, headaches, nausea, vomiting, & blurred vision Median survival 1 year with treatment 2-4 weeks without treatment Long term survival 20% of AML patients 25% of AML children Complete remission 75% of children Chronic fatigue, reduced exercise capacity, fever, night sweats, bone pain, easy bruising, petechiae, abdominal fullness, early satiety with weight loss Dependent on stage of disease Mean survival 6 years with treatment Initial remission 90% Chronic fatigue, reduced exercise capacity, fever, night sweats, bone pain, easy bruising, petechiae, abdominal fullness, early satiety with weight loss Dependent on stage of disease Median survival 3 years with treatment Initial remission 90% Diagnostic Procedures: Sudan black or myeloperoxidase, Cytogenic analysis, Lumbar Puncture Treatment: Chemotherapy Drugs used to destroy cancer cells or stop their growth to either slow down the disease or to bring it into remission (no disease signs) 3 Phases: induction, consolidation and maintenance Causes: Katie Davis, Michelle Eads, Kate Jamis, Joe Kussman, Michelle Robbins, TC Roope, & Becky Seifert Low numbers of white blood cells that can increase risk for infection Low numbers of red blood cells cause anemia. Low numbers of platelets can cause bruising and bleeding. Side effects: Loss of hear, hunger, & weight, Sores in the mouth & throat, Nausea & vomiting, Constipation, Diarrhea, Tiredness (fatigue), Numbness of fingertips or toes caused by damage to the nervous system, Damage to the heart, liver or kidneys, Delays in growth & learning ability in some kids, Infertility, Risk of getting another kind of cancer later Treatment: Radiation Most patients do not receive radiation therapy However, children who have signs of disease in the central nervous system (brain and spinal cord) or have a high risk of the disease spreading to this area may receive radiation therapy to the brain Treatment: Bone Marrow Transplant Allogenic Autologous (Foley) Common Metastatic Sites Because the cells are blood borne they can go to any site or organ Liver, Spleen, Lymph Nodes, CNS, Kidneys, Gonads Exercise Program Research shows that kids undergoing treatment for ALL participate in significantly less moderate to vigorous physical activity every week than their healthy peers. Children participating in aerobic and resistance training showed significant increases in aerobic fitness, strength, and functional mobility. Physical therapy initiated early in treatment and with emphasis on endurance activities may improve quality of life and stamina. Exercise Program: Aerobic Individual doses depending on degree of sickness and phase of chemotherapy Light levels of activity during induction and consolidation (similar to phase I cardiac rehab) Light-moderate activity on some days of the week during maintenance phase Large muscle group activity (bike riding, swimming, playing soccer) Working up to the guidelines and recommendations for healthy kids At least 60 minutes & up to several hours of moderate to vigorous activity 5-7 days/week (several 15 min sessions) Discourage periods of inactivity > 2 hours during the day Exercise Program: Strength Muscle strength and endurance training 3 days/week using exercise balls, medicine balls, and resistance bands (be creative!) Emotional Aspect Educate patient/parents Support child Keep normalcy in life – rules, chores, etc. Support Groups For family members/caregivers, peer-to-peer as kids get older Fatigue Make sure pt is having fun at therapy. Keep treatment fun, like with any child. Back to school program Info for school nurse, etc to help child adjust to school healthily. Helps get child prepared to go back to school. Emotional, physical, and cognitive effects of dealing with cancer/cancer treatment. Make-a-Wish foundation, etc. Children’s Hospice Centered on child-based end of life care Survival Rates Five year relative survival rates for ALL in children < 15 years old Prognosis Negative Factors Male, > 10 years old, Burkitt’s Type (B-cell) classification of ALL, B-cell immunophenotype, White Blood Cell count >100,000, CNS involvement, Cytogenetic abnormalities