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14 Cards in this Set
- Front
- Back
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peripheral smear
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abundant granulocytes, ranging from immature through mature
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tests to order
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peripheral blood FISH for BCR-ABL
RT PCR Ph chromosome t (9; 22) bone marrow biopsy |
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elevated neutrophil count due to severe infection
elevated leukocyte alkaline phosphatase score |
leukemoid reaction
leukocyte alkaline phophotase score is decreased in CML cytogenetic and molecular testing for CML is diagnostic |
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seen in infancy and childhood
Ph chromosome is negative |
juvenile myelomonocytic leukemia
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MPS
overproduction of maturing monocytic cells dysplatic changes in myeloid lineage absence of Ph chromosome |
chronic myelomonocytic leukemia
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blood disorders characterized by abnormal number of WBC, RBC, or platelets in blood and marrow
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myeloproliferative disorders (MPD)
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MPD subtypes
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chronic myelogenous leukemia (CML)-predominance of mature granulocytes
polycythemia rubra vera (PV)-excessive number of RBC essential thrombocytosis (ET)-excessive number of platelets primary myelofibrosis-extensive bone marrow scarring |
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MPD characteristics
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potential to evolve into AML or myelodysplasia:
CML-100 (if untreated) primary myelofibrosis: 5-25% PV-10% ET: 3-4 % overlap syndromes and evolution to other MPDs occurs |
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chronic myelogenous leukemia
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age at diagosis-greater than 60
primary manifestation is elevated circulation granulocytes w/ splenomegaly all stages of granulocyte maturation seen in peripheral blood |
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CML phases and symptoms
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symptoms due to splenomegaly and hypermetabolic state-LUQ pain, L flank pain; fevers, night sweats, wt loss
phases: chronic (stable): 3-5 years accelerated phase-6 months blast crisis phase (lymphoid or myeloid)-terminal if not treated |
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pathology
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stem cell lesion involves translocation of genetic material b/t chromosomes 9, 22
philadelphia chromosome-shortened chromosome 22 c-ABL oncogene (on 9) translocation to BCR (on 22) new protein produced w/ unregulated tyrosine kinase activity BCR-ABL produces phenotype in stable phase CML |
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CML treatment
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untreated CML terminates in blast crisis (AML or ALL) unless treated w/ allogenic bone marrow transplantation
Imatinib (1st generation TKI) Dasatnib and Nilotinnib (2nd gen tyrosine kinase inhibitors) targets BCR-ABL fusion protein-excellent long term disease free survival imatinib resistance treatable w/ 2nd generation TKIs |
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Pharmacology: tyrosine kinase inhibitors
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imatinib mesylate
dasatinib MOA-inhibit tyrosine kinase enyzme and cell proliferation clinical uses: imatinib (oral)-CML 1st line defense dastinib-for imitinib resistance |
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TKI side effects
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imatinib-NVD, edema, pleural effusion
dasatinib-myelosuppressive |
