Myeloid Neoplasms Flashcards
What are 3 morphological features of AML?
- At least 20% of bone marrow is immature blasts
- Blasts have Auer rods
- Marrow fibrosis
How is AML differentiated from ALL?
Staining for myeloid specific antigens.
What are some clinical features of AML?
Fatigue, fever and spontaneous bleeding
What chromosomal translocation can cause AML?
t(8:21) and inv(6). These disrupt the CFB1 alpha and beta genes. The transcription factor is needed for normal hematopoiesis.
Discuss the AML subtype t(15;17)
This translocation causes the production of a fusion gene (RAR-PML) that interacts with transcription factors resulting in an inhibition of maturation.
What treats AML subtype t(15:17)
All trans retinoid acid; ATRA
What are the hallmarks of MDS?
- Progenitors undergo apoptotic cell death
- Bone marrow is taken over my mutated progeny of neoplastic multipotent stem cell
- Dysplastic differentiation amongst cell types.
What is the hallmark of a Myeloproliferative disorder?
A mutated and constantly activated tyrosine kinase
Discuss the pathogenesis of CML?
A reciprocal translation (Philadelphia Chromosome) produces a mutated BCR-ABL gene directs a constitutively active tyrosine kinase
Morphology of CML?
- Macrophages with blue-green cytoplasm.
- Hypercellular marrow
- Blood predominant with Myelocyte series
What is Polycythemia Vera?
Increased production of RBCs, platelets and granulocytes; Associated with mutations in the tyrosine kinase JAK2.
Clinical features of PCV?
- Increased Hematocrit
2. Increased Total blood volume
Morphology of PCV?
- Hypercellular marrow WITH FAT
2. Peripheral blood contains basophils and large platelets
What is Essential Thrombocytosis?
Increased production of platelets; Associated with mutations in JAK2 and MPL tyrosine kinases.
How do you distinguish ET from PCV and Primary Myelofibrosis?
Absence of polycythemia and marrow fibrosis respectively.
