Flashcards in Pathoma Chapter 6: Part 2 Deck (51)
What are myeloproliferative disorders (MPDs)?
neoplastic proliferation of mature cells of myeloid lineage
When do MPDs usually present?
late adulthood (50-60 y/o)
T or F. in MPDs cells of all myeloid lineages are increased.
T. Subtypes are classified based on the major cell type produced
Complications with MPDs include what?
- increased risk for hyperuricemia and gout due to high turnover of cells
- progression to marrow fibrosis or transformation to acute leukemia
What are some types of MPDs?
- chronic myeloid leukemia (CML)
- polycythemia vera (PV)
- essential thrombocytopenia
What is CML caused by?
neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors
What is characteristically increased in CML?
Cause of CML?
t(9,22) Philadelphia chromosome which generates a BCR-ABL fusion protein with increased tyrosine kinase activity
Treatment for CML?
Imatinib, which blocks tyrosine kinase activity
Progression of CML?
-splenomegaly common and enlarging spleen suggest progression to accelerated phase of the disease and can transform to acute leukemia (ALL 1/3 of times and AML 2/3 of times- because mutation is in a pluripotent stem cell) shortly after
How is CML distinguished from a leukomoid reaction (reactive neutrophilic leukocytosis)?
1) Negative leukocyte alkaline phosphatase (LAP stain) in CML
2) Increased basophils in CML
3) t(9,22) presence
What is polycythemia vera (PV) caused by?
proliferation of mature myeloid cells- especially RBCS (granulocytes and platelets also increased)
Clinical symptoms of PV?
due to hyper viscosity of blood:
1) blurry vision and headache
2) flushed face due to congestion
3) itching after bathing (histamine mediated)
4) risk of venous thrombosis
Treatment for PV?
without treatment, death usually occurs in 1 year
How is PV distinguished from reactive polycythemia?
1) In PV, EPO levels are decreased and SaO2 is normal
2) In reactive polycythemia, SaO2 is low and EPO is increased
What is essential thrombocythemia (ET) caused by?
neoplastic proliferation of mature myeloid cells, especially platelets
PV and ET (and myelofibrosis sometimes) are both associated with what?
Jak2 kinase mutation
T or F. ET commonly progresses to acute leukemia
F, hardly ever progress and no significant risk for hyperuricemia or gout
What is myelofibrosis caused by?
neoplastic proliferation of mature myeloid cells, especially megakaryocytes
What is the root cause of marrow fibrosis in myelofibrosis?
megakaryocytes produce excess platelet- derived growth factor (PDGF)
What is common on a smear in myelofibrosis?
tear drop RBCs
Painful LAD is indicative of what?
Painless LAD is indicative of what?
- chronic inflammation
- metastatic carcinoma
What are lymphomas caused by?
noeplastic proliferation of lymphoid cells that forms a mass; may arise in lymph node or extranodal tissue
Subtypes of lymphoma?
-Hodgkins (40%) and non-Hodgkins (60%)
NHL is further divided based on what?
cell type (B vs T cell), pattern of cell growth expression of surface markers, and cytogenetic translocations
Subtypes of NHL?
1) small B cell (many types)
2) intermediate sized B cell (Burkitt lymphoma)
3) large B cell (diffuse large B-cell lymphoma)
High yield subtypes of small B cell NHL?
- follicular lymphoma
- mantle cell lymphoma
- marginal zone lymphoma
- small lymphocytic lymphoma
What is follicular lymphoma?
neoplastic proliferation of small B cell (CD20+) that form follicle-like nodules