MDS and MPN Flashcards
What is MPN?
Proliferation of myeloid cells of abnormal growth.
What are features of CMML?
Hypersegmentation of monocytes
What are the features of eosinophilic leukemia?
eosinophils with dysplastic changes
- elevated maturation of eosinophils
Which mutation is common in PV PMF and ET?
JAK2
What is the difference between relative polycythemia and absolute polycythemia?
Relative is not actually PV, increased RBC due to dehydration, vomiting or sport doping
Absolute PV is divided by primary and secondary, increase in RBC caused by true PV (Mutation, other diseases)
What are some treatments for PV?
- Therapeutic phlebotomy
- aspirin
What causes polycythemia vera (PV)>
defect in the pluripotential stem cell.
What is PMF
Primary myelofibrosis is replacement of normal BM elements with fibrotic connective tissue
What are key findings of PMF?
- Dacrocytes: Extramedullalry hematopoiesis
- Leukoerythroblastic
-megathrombocytes - BM : dry tap
What is essential thrombocythemia (ET)?
Increased proliferation of megakaryocytes in the marrow at the multipotential stem cell
What are findings of ET?
Marked thrombocytosis , clusters of PLT in PBF and BM
BM:
- increased cellularity
- Giant PLT
- abnormal megakaryocytes
What is mastocytosis?
Neoplastic proliferation of mast cells in one or more organs.
Which mutation is related to mastocytosis?
KIT mutation
What is the difference in Mast cell leukemia and Mast cell sarcoma?
leukemia: >10% immature mast cells in PBF and >20% in BM
Sarcoma: solid mast tumor
What is myelodyplastic syndrome?
- BM malignancy, poorly formed cells with increased marrow destruction.
What are the two subtypes of MDS?
- Primary: new case, unrelated to other treatment
- Secondary : caused by prior therapy
what are features of MDS in terms of diagnosing it?
- Leukoerythroblastic
- megaloblastic
- may have ringed sideroblast
BM: - hypercellular + immature - pancytopenia
What dysplastic changes can be seen in the nucleus of cell in MDS?
- multinuclear
- disintegration of nucleus
- hyper-segmentation
- hypo-segmentation
- nuclear budding
- ringed sideroblast
What cytoplasmic dysplastic changes can be seen in MDS?
- abnormal vacuolation
- Poor granulation (Hypo/hyper)
- poor staining (Harlequin)
- hypogranular thrombocytes
