acute leukemia
Terms
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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
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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 - 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)