Chronic Leukemias Flashcards
Which translocation is found in CML?
t(9;22) Philadelphia chromosome
True or false
the Philadelphia chromosome has a good prognosis in AML and a bad prognosis in CML
False- good prognosis in CML, bad prognosis in AML
What are the clinical features of CML?
fatigue
weight loss
sweating
painless lymphadenopathy
massive splenomegaly > 15 cm below costal margin
What is seen on the peripheral blood film in CML?
normocytic, normochromic cells
increased uric acid
increased LDH
WBC: 10,000 - 600,000
leukemic cells
basophilia
Basophilia occurs as a result of blast crisis in CML. What are the clinical features resulting from basophilia?
diarrhea, pruritus, flushing
Which tests definitively diagnose CML?
Cytogenetics and BM biopsy
What are the side effects of imatinib?
nausea
fluid retention (e.g. periorbital oedema, CHF)
prolonged QT interval on ECG
muscle cramps
rash
myelosuppression
What are the side effects of dasatinib?
myelosuppresion especially thrombocytopenia
CHF
pulmonary HTN
prolonged QT interval
In CML with T3151 mutation which TKI drug is given?
ponatinib
Which type of leukemia presents with lymphocytosis with smudge cells?
CLL
Which stage in the Rai System has lymphocytosis + hepatosplenomegaly?
Stage 2
True or false
A 13q deletion in CLL has a poor prognosis
False: favorable prognosis
What is the main cause of death in CML?
Transformation to a blast phase
What is the clinical presentation of CML?
hyper metabolism symptoms (weight loss, lethargy, anorexia or night sweats)
splenomegaly
anaemia symptoms (pallor, dyspnea, tachycardia)
abnormal platelet function
gout or renal impairment
What is the main treatment for CML?
Tyrosine kinase inhibitors
What are the lab findings in CML?
leucocytosis (main feature)
increased circulating basophils (characteristic feature)
normocytic, normochromic anaemia
platelet count mostly increased (may be normal or decreased)
hypercellular BM (granulocytic predominance)
BCR-ABL1 gene fusion
increased serum uric acid
Which TKI is associated with the highest risk of thrombotic events?
ponatinib
Which treatment is used for CML in pregnancy?
a-inteferon
What are the features of the accelerated phase in CML?
anaemia
thrombocytopenia
basophilia (>20%)
marrow blast cells (10-19%)
spleen may be enlarged
marrow may be fibrotic
new clonal chromosomal or molecular abnormalites may appear
What is acute tranformation in CML?
transformation into acute leukemia (mainly AML)
