Chronic Leukemia Flashcards
What is chromic myelogenous leukemia (CML)?
Clonal stem cell disorder with increased proliferation of myeloid elements at all stages of differentiation
Myeloproliferative neoplasia
Only known etiology is ionizing radiation exposure
Median age of presentation is 50
Slight male predominance
What is the pathogenesis of CML?
Translocation t(9:22)- Philadelphia chromosome forming BCR-ABL gene Disregulates apoptosis
What are the lab findings in CML?
Leukocytosis (150,000 mean)
Basophilia
How is CML treated?
Allogenic stem cell transplant- must have donor, be in good health and have money
Imatinib (Gleevec)- bcr-abl tyrosine kinase inhibitor- first targeted therapy
Alpha interferon
Hydroxyurea
What is chronic lymphocytic leukemia (CLL)?
Malignancy characterized by the accumulation of non proliferating mature lymphocytes
What are the clinical features of CLL?
Disease of the elderly (72 median age) Affects men more than women Often asymptomatic Lymphadenopathy is commom Splenomegaly Infections with low IgG levels
How is CLL diagnosed?
Typical CBC with absolute lymphocyte count >10,000
Flow cytometry: CD5,CD19, 20, 23
Smudge cells on peripheral smear
How is CLL treated?
Staging is based on lymphocytosis and lymphadenopathy
Takes years to develop
Most patients do not require therapy at diagnosis
Monitor for rapid rise in WBC
What is hairy cell leukemia ?
Rare, indolent B cell lymphoproliferative disorder
Affects mostly men
Unknown etiology
Pathologie features are CD103,123 are bright on immunophenotype
What are the clinical features of hairy cell leukemia ?
Splenomegaly Anemia Thrombocytopenia Neutropenia Monocytopenia Dry tap on bone marrow
How is hairy cell leukemia treated?
Treatment indications are fatigue, recurrent infections and physical discomfort due to splenomegaly
Splenectomy
If no indication for treatment consider observation