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Final Exam
Chapter 24: Acute Myeloid Leukemia
39
Science
Graduate
04/10/2013

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Term
Acute Leukemia
Definition
acquired somatic mutation in hematopoietic stem cells or progenitor cells which confers a proliferative or survival advantage and properties of limitless self-renewal to those cells and impairs hematopoietic differentiation
Term
Acute myeloid leukemia
Definition
somatic defect primarily affects common myeloid precursor
Term
Acute lymphoblastic leukemia
Definition
somatic defect primarily affects common lymphoid precursor
Term
Myelodysplastic syndrome
Definition
genetic defect which is characterized by cytopenias and dyshematopoiesis in all cell lines
Term
Common variants of AML as defined by WHO classification
Definition
AML with recurrent genetic abnormalities, AML with multilineage dysplasia, AML and MDS-therapy-related, and AML not otherwise categorized
Term
AML with recurrent genetic abnormalities definition by WHO classification
Definition
translocations, acute promyelocytic leukemia with translocations, AML with abnormal bone marrow eosinophils and translocation, and AML with 11q23 abnormalities
Term
AML with multilineage dysplasia definition by WHO classification
Definition
can follow MDS or MDS/MPD, +/- prior myelodysplastic syndrome
Term
AML and MDS-therapy-related definition by WHO classification
Definition
alkylating agent related, topoisomerase type II inhibitor-related, or other types
Term
AML not otherwise categorized by WHO classification
Definition
AML minimally differentiated (M0), AML without maturation (M1), and AML with maturation (M2), acute myelomonocytic leukemia (M4), acute erythroid leukemia (M6), acute megakaryocytic leukemia (M7), acute basophilic leukemia, acute panmyelosis with myelofibrosis, and myeloid sarcoma
Term
Typical blood picture for erythrocytes in AML
Definition
anemia often <10 g/dL, nRBCs, Howell-jolly bodies, basophilic stippling, and pappenheimer bodies
Term
Typical blood picture for leukocytes in AML
Definition
variable (1-200 x 103/uL), most typically seen 5-30 x 103/uL, blasts make up 15-95% of all WBCs, granulocytopenia and monocytopenia, eosinophilia and basophilia, dysplastic PMNs
Term
Typical blood picture for thrombocytes in AML
Definition
thrombocytopenia often <20 x 103/uL, giant platelets and hypogranular platelets
Term
Myeloid:Erythroid ratio in the bone marrow in AL
Definition
decreased ratio, indicated hypercellularity
Term
Typical results of MPO cytochemical stains in AML
Definition
Myeloperoxidase is positive for myeloblasts and negative for lymphoblasts, monoblasts and erythroblasts
Term
Typical results of SBB cytochemical stains in AML
Definition
Sudan black B stains lipids of cytoplasmic granules, it will be positive for myeloblasts and negative for lymphoblasts, monoblasts and erythroblasts
Term
Auer rods
Definition
rod-like filaments of aggregated primary granules
Term
Significance of Auer rods
Definition
their presence excludes the diagnosis of ALL because they are only seen in AML
Term
AML with recurrent genetic abnormalities presentation
Definition
Acute Promyelocytic Leukemia (APL) which usually occurs in younger patients and is responsive to all-trans retinoic acid. APL presents with promyelocytes and multiple Auer rods. In APL there is a t(15:17) which produces a PML-RARs fusion protein which blocks differentiation at the promyelocyte stage of development.
Term
AML with multilineage dysplasia presentation
Definition
This type of AML can present de novo or following MDS/MPD in which there is dysplasia in two or more myeloid cell lines. The peripheral blood shows hypogranular PMNs, pseudo-pelger huet cells, megaloblastoid RBCs, multinucleated nRBCs, and ring sideroblasts with micromegakaryocytes.
Term
AML and MDS-therapy related presentation
Definition
usually presents 5-6 years following an alkylating agent exposure in cytotoxic chemo or radiation therapy. The cytogenetic abnormalities are similar to those seen in AML with multilineage dysplasia and de novo MDS.
Term
AML not otherwise categorized presentation
Definition
greater than 20% blasts with clear evidence of maturation beyond promyelocyte stage. Acute Myelomonocytic leukemia (AMML) is included in this category because of proliferation of myelocytic and monocytic precursors coupled with an extramedullary disease.
Term
Patient history and type of leukemia
Definition
AML with t(15:17)(q22;q21) (PML/RARa) and variants is distinguished from other leukemias because it occurs in patients of younger age
Term
Physical assessment and type of leukemia
Definition
coagulopathy occurs in AML with t(15:17)(q22;q21) (PML/RARa) and variants, AML and MDS therapy related diseases occur in populations who had cytotoxic chemo and/or radiation therapy in that last 5-6 years
Term
Laboratory findings and type of leukemia
Definition
when abnormal promyelocytes and multiple cells with Auer rods predominate the leukemia is AML with t(15:17), when >50% of cells are dysplastic in two or more myeloid cell lines then the leukemia is AML with multilineage dysplasia, if there is >20% then the leukemia is AML with maturation
Term
Cellular presentations of AML relationship to prognosis
Definition
Best prognosis is with APL, the worst prognosis is with AML, AEL, or AMkL. In between is AML +/- maturation, AMML, and AMoL
Term
Cellular presentation of AML relationship to complications
Definition
Malignant progranulocytes in AML with t(15:17) can lead to DIC and thrombocytopenia. Acute myelomonocytic leukemia can lead to extramedullary disease like gingival hypertrophy, leukemia cutis, and meningeal leukemia
Term
Wright-stain morphology of AML with recurrent genetic abnormalities relationship to flow cytometry
Definition
CD13, CD33, CD34, MPO, and CD56+
Term
Wright-stain morphology of AML with recurrent genetic abnormalities relationship to genetic testing
Definition
Wright-stain morphology of AML with recurrent genetic abnormalities relationship to genetic testing:
Term
Wright-stain morphology of AML with multilineage dysplasia relationship to flow cytometry
Definition
the leukemic cell of origin is an hematopoietic stem which, and the CD markers are CD34, CD13, and CD33 +, and CD56 and CD17 variable
Term
Wright-stain morphology of AML with multilineage dysplasia relationship to genetic testing
Definition
cytogentic abnormalities indicate mutations, causes dysplasia in two or more myeloid cell lines
Term
Wright-stain morphology of AML and MDS-therapy related relationship to flow cytometry
Definition
immunophenotyping shows CD34, CD13, CD33 +, and CD56 and CD17 variable
Term
Wright-stain morphology of AML and MDS-therapy related relationship to genetic testing
Definition
unbalanced translocations or deletions involving chromosomes 5 and 7 or balanced translocations (topoisomerase II inhibitor therapy), refractory to treatment
Term
Wright-stain morphology of AML not otherwise categorized relationship to flow cytometry
Definition
proliferation of myelocytic (CD 13 and CD33+) and monocytic precursors (CD14, CD11b, CD11c and lysozyme +)
Term
Wright-stain morphology of AML not otherwise categorized relationship to genetic testing
Definition
varies
Term
Peripheral blood results in relation to complete or partial remission
Definition
normal blood picture returns
Term
Peripheral blood results in relation to relapse
Definition
monitor pancytopenia, severe granulocytopenia, and/or thrombocytopenia
Term
Peripheral blood results in relation to engraftment
Definition
only therapeutic choice that provides potential for prolonged for a disease free survival
Term
WHO classification of AML
Definition
bases on morphology, cytogenetic, molecular genetics, and immunologic markers, >20% of nonerythroid nucleated cells in BM are blasts
Term
FAB classifications of AML
Definition
based on morphologic and cytochemical evaluation, >30% of nonerythroid nucleated cells in BM are blasts
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