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17 Cards in this Set

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Myelodysplastic syndrome definition
maturation defects resulting in ineffective hematopoiesisclinical features myelodysplastic syndrome
Pathological findings myelodysplastic syndrome
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
Subtypes of myelodysplastic syndrome
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
Treatment of myelodysplastic syndrome
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.
Definition of leukemia
marrow based clonal malignant hematopoietic neoplasms
diagnosis of leukemia
finding blasts in peripheral blood and leukemic cells in bone marrow
concequences of marrow failure due to crowding in leukemia
anemia: pallor and lethargy thrombocytopenia: petechiae neutropenia: infection
organs infiltrated in leukemia
spleen, lymph nodes, liver, CSF testes, thymus (T-ALL), skin, orbit
histological features of blasts
high nucleus to cytoplasm ratio, fine chromatin, distinct nucleoli (sometimes). Vary in size from lymphocyte to >neutrophils.
stains for identifying myeloid blasts
cytochemical : Sudan black, myeloperoxidase, esterase. Wright’s stain for Auer rods
difference in immunology of AML vs ALL
AML: CD13, CD33, CD117, cMPO; ALL: TdT CD10. (B Cell: CD19, cCD79a; T Cell: CD2 CD7 cCD3)
Acute Myeloid Leukemia age
MDR-AML in older pts, de novo AML in 30s
de novo AML treatment
respond favorably to chemotherapy
acute promyelocytic leukemia genetics, tx
t(15;17)(PML/RARa) inactivates retinoic acid receptor a, can be reactivated by all-trans retinoic acid
Acute lymphoblastic leukemia clinical features
mediastinal mass. may have splenomegaly, hepatomegaly, lymphadenopathy. Childhood disease.
Treatment outcome and type of Acute lyphoblastic leukemia
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.
Immunophenotypes of Acute Lymphoblastic Leukemias
85% B (CD19 CD79a TdT); 15% T (CD2 CD7 cCD3 TdT) ; 1-2% like burkitt’s (CD19 CD10 CD20 surface Ig)