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17 Cards in this Set
- Front
- Back
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Myelodysplastic syndrome definition
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maturation defects resulting in ineffective hematopoiesisclinical features myelodysplastic syndrome
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Pathological findings myelodysplastic syndrome
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hypercellular marrow and peripheral cytopniea. Abnormal morphology including weird erythrocytes, bilobed nuclei in neutrophils (pelger-huet cells), big platelets w/ no granules; often genetic abnormalities shared w/ AML found on G banding
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Subtypes of myelodysplastic syndrome
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low grade: refractory anemia (normal blast count) with or without ringed sideroblasts, average survival 6 years.
High grade: refractory anemia with excess blasts; average survival 6 months often history of chemotherapy or radiation and often multiple cytogenic abnormalities |
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Treatment of myelodysplastic syndrome
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supportive w/ blood transfusions and antibiotics, only long term tx is bone marrow transplant. usually too old. must die from the cytopenias. 1/3 progress to AML.
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Definition of leukemia
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marrow based clonal malignant hematopoietic neoplasms
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diagnosis of leukemia
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finding blasts in peripheral blood and leukemic cells in bone marrow
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concequences of marrow failure due to crowding in leukemia
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anemia: pallor and lethargy thrombocytopenia: petechiae neutropenia: infection
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organs infiltrated in leukemia
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spleen, lymph nodes, liver, CSF testes, thymus (T-ALL), skin, orbit
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histological features of blasts
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high nucleus to cytoplasm ratio, fine chromatin, distinct nucleoli (sometimes). Vary in size from lymphocyte to >neutrophils.
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stains for identifying myeloid blasts
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cytochemical : Sudan black, myeloperoxidase, esterase. Wright’s stain for Auer rods
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difference in immunology of AML vs ALL
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AML: CD13, CD33, CD117, cMPO; ALL: TdT CD10. (B Cell: CD19, cCD79a; T Cell: CD2 CD7 cCD3)
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Acute Myeloid Leukemia age
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MDR-AML in older pts, de novo AML in 30s
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de novo AML treatment
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respond favorably to chemotherapy
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acute promyelocytic leukemia genetics, tx
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t(15;17)(PML/RARa) inactivates retinoic acid receptor a, can be reactivated by all-trans retinoic acid
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Acute lymphoblastic leukemia clinical features
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mediastinal mass. may have splenomegaly, hepatomegaly, lymphadenopathy. Childhood disease.
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Treatment outcome and type of Acute lyphoblastic leukemia
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T-ALL good prognosis. B-ALL t(12;21)(TEL/AML) good. t(9;22)(BCR/ABL), t(4;11)(MLL/AF4) or hypodiploidy bad.
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Immunophenotypes of Acute Lymphoblastic Leukemias
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85% B (CD19 CD79a TdT); 15% T (CD2 CD7 cCD3 TdT) ; 1-2% like burkitt’s (CD19 CD10 CD20 surface Ig)
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