ALL Flashcards
Epidemiology
Median age at diagnosis: 17
More common in Hispanics and White
Older age=poorer prognosis
Pathophysiology
Arise from single leukemic cell–> expands and acquires additional mutations–>proliferation resulting in monoclonal population of leukemia cells
Failure to maintain balance between proliferation and differentiation
Defect in the lymphopoietic stem cell or an early lymphoid precursor
Risk factors
Genetic: 4% of children with ALL carry a germ line cancer predisposition gene
Radiation
Viral infections: EBV and HIV
Presention
Anemia, thrombocytopenia, neutropenia
Bone pain
Lymphadenopathy
Gum hypertrophy
Abdominal masses
Painless testicular enlargement
Diagnosis
Bone marrow biopsy with > or equal to 20% blasts
Philadelphia Positive disease
25% of adult ALL patients will be Ph+
TKI added to multi-agent chemotherapy
Incorporation into therapy improves outcomes when given prior to allogeneic stem cell transplant
Treatment overview
Induction: goal is remission
Consolidation: goal eradicate residual disease
Maintenance: prevent relapse
Consolidation
Multiagent therapy
Blinatumomab
Allogeneic stem cell transplant
ALL CAN HIDE IN SANCTUARY SITES (BRAIN AND TESTES)
All patients should receive CNS prophylaxis or treatment
Ph positive
Age/comorbidities
Intensity of regimen may vary based on age/comorbidities
TKI + multiagent chemotherapy
TKI + steroids
TKI + blinatumomab
Maintenance: TKI + vincristine + prednisone
Ph negative AYA
Age/comorbidities
AYA–>pediatric inspired regimen–>multiagent chemotherapy
Ph negative 65+
Multiagent chemotherapy
Inotuzumab
Palliative steroids
Maintenance: weekly methotrexate + daily 6MP+ monthly vincristine
Ph negative < 65
Multiagent chemotherapy
HyperCVAD
Part A:
-Hyper-fractionated cyclophosphamide
-Vincristine
-Doxorubicin
-Dexamethasone
Part B:
-Methylprednisolone
-Methotrexate
-Cytarabine
Maintenance:
-6-MP
-Methotrexate
-Prednisone
-Vincristine
Methotrexate (high dose) and cytarabine
Intrathecal cytarabine and intrathecal methotrexate each cycle
Blinatumomab
BiTE: CD19 and CD3
SE: cytokine storm
Continuous administration for 4 weeks on and 2 weeks off
Cytoreduction when blast count is > 15,000
Asparaginase
