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Path: Myeloproliferative Neoplasms
Terms in this set (53)
List 4 examples of myeloproliferative neoplasms
1. chronic myelogenous leukemia
2. polycythemia vera
3. primary myelofibrosis
4. essential thrombocythemia
Cellular morphology in myeloproliferative neoplasms are usually normal, except for which cell?
Term that describes the hematopoiesis in myeloproliferative neoplasms
effective -- cells are able to enter the blood from the marrow
% blasts seen in the marrow of myeloproliferative neoplasms?
normal to slightly increased
Myeloproliferative neoplasms are all due to changes seen in what cells?
hematopoietic stem cells
Is organomegaly common in myeloproliferative neoplasms?
yes, spleen + liver
What signaling pathway is affected in all myeloproliferative neoplasms?
Predominant lineage of cells increased in CML
Predominant lineage of cells increased in polycythemia vera
RBCs (erythroid cells)
Predominant lineage of cells increased in essential thrombocythemia
What are the 3 phases seen in all myeloproliferative neoplasms?
1. chronic phase
2. accelerating phase
3. acute blast phase (or marrow failure)
In the marrow, what kind of cells would you expect to find in a patient with a more severe stage of a myeloproliferative neoplasm?
increased blast number
-- due to failure of maturation
Chromosomal abnormality that causes CML and gene product
t(9;22); BCR-ABL fusion gene
Tyrosine kinase gene in CML that has been translocated
note: the ABL is the reason for the proliferation, BCR is the reason for continued expression of ABL
Tyrosine kinase mutation seen in most myeloproliferative neoplasms
note: seen in PV, PMF and ET so is non-specific
Molecular target therapeutic agent used for CML
-- small molecule inhibitor of ABL tyrosine kinase (TKI class)
Patient age for CML
Fatigue, Weight Loss Anemia, Sweats, Splenomegaly
List 3 cell types you'd expect to see increased in the peripheral blood of a patient with CML
2. myelocytes (immature neutrophils)
-- absolute basophilia = buzzword for CML
Change in neutrophil alkaline phosphatase in a Pt with CML;
(First Aid) what does this help to differentiate it from?
FA: leukemoid reaction, which has an increase in leukocyte alkaline phosphatase
Megakaryocyte change seen in marrow of a Pt with CML
What organ change is seen in the accelerated phase of CML?
Transformation of CML into an acute leukemia is commonly termed a:
blast phase/ crisis
% blasts seen in blast phase of CML
Why is it possible to develop ALL (acute lymphoblastic leukemia) in a patient with CML (a myeloproliferative disease)?
it is a disease of hematopoietic stem cells, which is a shared lineage with lymphocytes
What is required for the Dx of CML?
Philadelphia chromosome t(9;22)
Gender predominance in polycythemia vera
-- mean age 60 years, so in line with other MPDs
what are 3 stages of PV?
List 3 common complaints in patients with polycythemia vera
1. blurry vision
-- all due to increased viscosity of blood
Mesenteric, portal or splenic vein thromboses are commonly associated with what disorder?
Serum levels of _____ should be evaluated to make a Dx of Polycythemia vera
-- should be LOW b/c of negative feedback
primary cause of death in pt. w/ polycythemia vera
thrombosis or hemorrhage
What effect does chemo have on polycythemia vera?
increases leukemic transformation, better to remove blood volume (essentially bloodletting)
During which stage of the disease is a red cell mass useful for Dx of polycythemia vera?
proliferative polycythemic phase (mass is increased)
The polycythemic stage of PV will have normocytic, norm chromic erythrocytosis unless:
the marrow runs out of iron
(de facto Iron deficiency due to increased demand)
Megakaryocyte change seen in polycythemic stage of PV
large megakaryocytes that are pleomorphic
What could be used, besides a bone marrow biopsy, to determine that a patient with PC has moved into the spent phase of the disease?
normalizing red cell mass that progressively decreases
leukoerythroblastosis picture in blood
What marrow changes are seen in the spent phase of PV?
fibrosis and myeloid metaplasia
-- this accounts for the leukoerythroblastosis and tear drop cells
Tear drop RBCs are associated with which phase of PV?
-- due to marrow fibrosis
By the end of the spent phase of PV, where is most hematopoiesis occurring? Why?
liver and spleen; myelofibrosis of the bone marrow
what are most common causes of death in PV?
thrombosis & hemorrhage
what growth factor are PV RBCs hypersensitive towards?
symptoms of primary myelofibrosis
fatigue, weight loss, sweatingm hyperuricemia, hepatosplenomegaly, anemia, thrombocytosis
Most common genetic mutation associated with primary myelofibrosis
JAK2 V617F (50%)
NO Philadelphia chromosome
what is another name for prefibrotic stage of PMF?
2 most common malignant causes of splenomegaly
1. primary myelofibrosis
2. hairy cell leukemia
Tear drop RBCs are most commonly associated with what myeloproliferative disorder?
note: also seen in the spent phase of polycythemia vera
During the fibrotic stage of primary myelofibrosis, what part of the bone marrow still has active hematopoesis?
osteosclerosis is characteristic of what stage of PMF?
Which myeloproliferative disorder may be seen in females around 30 y.o?
In patient with symptoms of essential thrombocythemia, what are the 2 most common presentations?
-- due to ineffectiveness of platelets produced
Megakaryocyte change seen in essential thrombocythemia
large giant mature megakaryocytes
-- but not bizarre forms
what therapies can worsen survival in essential thrombocythemia?
cytotoxic therapy (transformation to MDS or AML)
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