Chronic Leukemias Flashcards
What is the main distinguishing feature between chronic leukemia’s and acute leukemia’s
The chronic leukemia’s are distinguished from acute leukemia is by the slower progression
They are also more difficult to cure
What are the two subdivisions of chronic leukemia’s
myeloid and lymphoid groups
Define chronic myeloid leukemia
This is a clonal disorder of pluripotent stem cell
This is a clonal disorder of pluripotent stem cell
Chronic Myeloid Leukemia
Which disease accounts for 15% of leukemia’s
Chronic myeloid leukemia CML
Chronic Myeloid Leukemia (CML) accounts for what percentage of Leukemias?
15%
The diagnosis of chronic myeloid Leukemia is rarely difficult and is assisted by the characteristic presence of what
Ph chromosome
True or false the PH translocation in Chronic Myeloid Leukemia is also seen in Acute lymphoblastic Leukemia
True
Chronic Myeloid leukemia occurs most frequently between ages
Between the ages of 40 and 60 yrs old
True or False
Chronic Myeloid Leukemia can occur in children, neonates and the very old
True
List five clinical features of Chronic Myeloid Leukemia
Symptoms related to hypermetabolism (weight loss, lassitude, anorexia, night sweat)
Splenomegaly
Features of anemia
Signs of abnormal platelet function (bruising, epistaxis, menorrhagia, haemorrhage)
Gout or Renal impairment from Hyperuricaemia (from excess purine breakdown)
List five laboratory findings in chronic myeloid leukocytosis
Leukocytosis is usually >50 x 10^9 Increased circulating basophils Normochromic, normocytic anemia Platelet count increased (most frequently), may be normal or decreased Neutrophil alkaline phosphatase low
What is the basophil count in chronic myeloid leukemia
Increased
What is the color and size of red blood cells in chronic myeloid leukemia
Normochromic
Normocytic
What is the first line drug in management of chronic myeloid leukemia
Imatinib (Glivec) tyrosine kinase inhibitor
How does Imatinib (Glivec) work
Specific inhibitor of the BCR-ABL fusion protein and blocks tyrosine kinase activity by competing with ATP binding
What is the dosage of Imatinib administered to pts with Chronic Myeloid Leukemia
400mg/day
List some side effects of Imatinib
Skin rash
Fluid retention
Muscle pains
Nausea
(Sometimes neutropenia and thrombocytopenia)
What is done If neutropenia occurs during treatment of CML with Imatinib
Granulocyte Stimulating Factor
Define a complete cytogenetic response to treatment for Chronic Myeloid Leukemia
The absence of PH positive metaphases on cytogenetic analysis of the bone marrow
Patients with sub optimal response to treatment for chronic myeloid leukemia can be identified as those who
Fail to achieve a complete hematological response after three months
Failed to achieve any significant cytogenetic response in bone marrow after six months
Fails to achieve a major cytogenetic response in bone marrow which is less than 25% Ph+ metaphases by cytogenetic analysis after 1 year
Eventhough it is unclear how patients who have suboptimal response to chronic myeloid leukemia treatment should be treated, what are the potential options
Increase in dose of Imatinib to 600-800 mg/day
Or
Substitution or addition of second generation tyrosine kinase inhibitors DASATINIB or NILOTINIB (which can ivercome resistance to Imatinib)
How do you test for acquired resistance to Imatinib in pts treated for Chronic Myeloid Leukemia
Molecular Screening for mutations in the BCR-ABL fusion gene
What drug is used in the initial phase of treatment for Chronic Myeloid Leukemia CML to treat gout and prevent hyperuricaemia
Allopurinol
What is the purpose of Allopurinol in treatment of CML
used in the initial phase of treatment for Chronic Myeloid Leukemia CML to treat gout and prevent hyperuricaemia
Second line therapy for pts unable to take Imatinib in treatment for CML have a poor response, name two
Chemotherapy: Hydroxyurea, Busulfan
Alpha Interferon:
Describe the administration of Chemotherapy: Hydroxyurea treatment for CML
1.0-2.0g/day and then reduce in weekly increments to maintain 0.5-1.5g/day
(Busulfan only used in pts intolerant of Hydroxyurea)
Allogenic Stem Cell Transplantation (SCT) is the only established curative treatment for CML but, because of the risk, it is usually reserved for Imatinib failures.
Read
Only pts under what age can tolerate SCT
Under 65yrs
True or False
CML usually shows an excellent response to Imatinib in the chronic phase
True
