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18 Cards in this Set
- Front
- Back
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Median Age of AML
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65yrs. Less than 50% cured
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How is it distinguised?
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Immunophenotyping
Cytogenics Molecular Studies Bone marrow aspirate- AML when >20% of nucleated cells are blasts |
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Cause?
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NO CLEAR CAUSE
Secondary AML: Pre existing myelodysplasia Myeloproliferative disease Previous chemo Radiation Benzene exposure Congenital chromosomal abnormalities |
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What are the Class l mutations associated with AML?
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FLT3-1TD or FLT3- TKD
N or K Ras mutation = proliferation or survival advantage. Do not affect differentiation |
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What are the Class ll mutations associated with AML?
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PML or RARa
AML1 or ETO CBF beta or SMMHC NPM1 = Impairs haematopoietic differentiation therefore there is an increased population of immortal cells |
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What are the clinical features?
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Marrow Failure: Anaemia/ Thrombocytopenia/ Neutropenia= Fever, Infection and ulcers
Organ Infiltration: Bone pain/ Gum hypertrophy/ skin infiltration/ Leucostasis/ Splenomegaly/ CNS involvement |
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Diagnosis
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FBC= Decreased Hb/ Abnormal WBC/ neutropenia
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Treatment
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Supportive: RBC/ Platelet transfusions/ Antibiotics/ Anti- sickness medication/ Allopurino/ Rasburicase
Chemo: Intensive/ Non intensive |
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Prognosis
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Unfavourable: >60yrs/ Increased WBC/ Abnormalities of chromosome 5 or 7/ FLT3 ITD mutations/ secondary AML/ Refractory disease after chemo
Favourable: <60yrs/ Low WBC/ t(5:17)/ t(8:21)/ inv(16)/ NPM1 Mutation/ de novo/ Complete remission after chemo |
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Treatment according to risk of relapse
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Allogenic stem cell transplant
Graft vs Leukaemia effect (replacement of immune system) Risk: Acute rejection/ infection/ graft vs host disease This treatment is for when in 2nd remission or there is an increased risk of disease in 1st remission |
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What is chronic Myeloid Leukaemia?
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Clonal disorder of the haemopoietic stem cell
Increased myeloid proliferation= over production of neutrophils and their precursors |
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Characteristics?
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Slow progression
Median Age 50 yrs 3 stages Rare More likely in men |
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Mutation associated with CML
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Single genetic lesion (BCR/ABL) (ABL= oncogene) on the Philadelphia chromosome (1960)
Reciprocal translocation- Long arm of 9 and 22 Fusion of breakpoint cluster region |
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What does the mutation cause?
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Increased tyrosine kinase activity which increases proliferation of mature and immature myeloid cells in marrow
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Symptoms
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Asymptomatic
Hypermetabolic symptoms: Fatigue/ sweats/ weight loss Splenomegaly Gout Leucostasis Anaemia symptoms Bleeding due to abnormal platelt function Constant erection |
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Diagnosis
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FBC= Increased WBC/ Increased Basophils/
Maybe decreased Hb and Increased platelet count Left shift with metamyelocytes Cytogenetics- FISH/ PCR |
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What are the phases of CML?
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Chronic phase: median 5-6yr stabilization
Accelerated phase: Median duration 6-9 months Blast crisis: Median survival 3-6 months |
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Treatment
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Imatinib Mesylate (Glivec)
Tyrosine Kinase inhibitor: Prevents ATP binding to BCR-ABL Given orally. Well tolerated (400mg) 80% achieve complete cytogenetic response |
