myeloproliferative disorders Flashcards
What are Myeloproliferative Neoplasms (MPN)?
Collection of disorders characterized by proliferation of cells with myeloid origin
MPNs include conditions such as Chronic Myeloid Leukemia and Polycythemia Vera.
What are common laboratory findings in CMML?
Leukocytosis with monocytosis, anemia, and thrombocytopenia.
What are the three main types of MPN that likely have the JAK2 V617F mutation?
- Polycythemia Vera
- Primary Myelofibrosis
- Essential Thrombocythemia
What is the most common cause of secondary polycythemia?
Increased EPO due to hypoxia.
What characterizes Primary Myelofibrosis (PMF)?
Marrow fibrosis and increased fibroblast activity.
What do mast cells contain?
Histamine and heparin.
True or False: Myeloproliferative Neoplasms can progress to Acute Myeloid Leukemia.
True.
What are the diagnostic methods for Mastocytosis?
- Bone marrow biopsy
- Skin biopsy
Bone marrow biopsy is the primary diagnostic tool.
What is Myelodysplastic Syndrome (MDS)?
Malignancy of the bone marrow with impaired maturation of blood cells
It involves the cell production of all cell lines.
What is the difference between Myelodysplastic Syndromes (MDS) and Myeloproliferative Neoplasia (MPN)?
- MPN: increased cell production
- MDS: impaired maturation
What factors categorize Myelodysplastic Syndrome?
- Number and type of blasts in BM and PB
- Type of dysplasia
- Presence/absence of ringed sideroblast
- Genetic testing
What characterizes MDS with ring sideroblasts (MDS-RS)?
Erythropenia with >15% ringed sideroblasts
Rare and rarely develops to AML.
What is unclassifiable MDS (MDS-U)?
Default classification for MDS that do not fit other categories
What are the cytoplasmic changes in dysplastic cells?
- Abnormal vacuolation
- Poor granulation
- Hypogranulation
- Hypergranulation
- Poor staining of granules
Harlequin cells are an example.
What is the typical cell size in dysplastic changes?
- RBC: macrocytic
- WBC: large polymorphs
- PLT: megakaryocytes or megathrombocytes
