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44 Cards in this Set
- Front
- Back
What do we call an accumulation of mature myeloid cells? |
Myeloproliferative disorder
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If RBCs are overproduced, what do we call that?
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Polycythemia vera
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If granulocytes are overproduced what do we call that?
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CML
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Of megakaryocytes or platelets are overproduced, what do we call that?
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Essenetial thrombocytemia
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True or false: In myeloproliferative disorders, you get a proliferation of one cell lineage at a time.
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False. Actually, it involves every single lineage. However, we name it based on the predominant cell type.
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What age group do we see myeloproliferative disorders in?
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it is a disease of late adulthood
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What do we see in marrow and on count in myeloproliferative disorders?
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Results in high WBC count (granulocytes) and hypercellular marrow
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Complications that are common in myeloproliferative disorders include?
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(1) Increased risk for hyperuricemia and gout (lets say you overproduce RBCs, the marrow spits out all the nuclei and those nuclei has to be degraded)
(2) Progression to marrow fibrosis |
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Progression to marrow fibrosis is called the?
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Spent phase
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Many myeloproliferative disorders can progress to what?
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Acute leukemia (accumulating mutations)
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CML is proliferation of what?
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Neoplastic proliferation of mature myeloid cells, especially granulocytes
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What cell type is characteristically increased in CML?
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Basophilia
CML is also the only leukemia with thrombocytosis. |
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This might be?
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Chronic myeloid leukemia, look at all the neutrophils, a myeloid precursor cell in the bottom and basophil.
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CML is driven by what?
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t(9;22), a BCR-ABL fusion gene
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First line treatment of CML is what?
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Imatinib, which blocks tyrosine kinase activity.
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In CML, _____________ is common. What phase does this correspond to?
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Splenomegaly.
Chronic phase. |
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You can divide CML into three phases, what are they?
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(1) Chronic phase
(2) Accelerated phase (3) Transformation phase (acute leukemia) |
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A man with CML has had stable splenomegaly for some time. All of a sudden it enlarges. What phase does this correspond to?
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Accelerated phase
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When does transformation in CML occur?
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Usually shortly after accelerated phase
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Transformation, when it occurs in CML can result in either ________ or _________.
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AML (2/3) or ALL (1/3)
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What cell proliferates in CML?
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Because CML can transform into either ALL or AML, it must be at the level of the HSC.
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What is a leukemoid reaction?
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Physiologic response to stress or infection
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On a smear, you observe a bunch of granulocytes and early percursors. What is your differential diagnosis?
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(1) Acute infection
(2) CML |
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How can you make the distinction between a leukemoid reaction and CML?
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(1) CML granulocytes are LAP negative (secondary granules)
- Cells of CML have no intention of fighting an infection, they are basically having a party. That's why LAP is negative. (2) CML is associated with increased basophils. (3) CML granulocytes exhibit t(9;22) |
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What is polycythemia vera?
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Myeloproliferative disorder with neoplastic proliferation of mature myeloid cells, especially RBCs.
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What cells are increased in polycythemia vera?
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RBCs especially, but also granulocytes and platelets.
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Polycythemia vera is associated with a ________ mutation.
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JAK2 kinase mutation
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Clinically, patients with PV are going to get symptoms of what?
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Symptoms of hyperviscosity.
(1) Blurry vision, headache (2) Increased risk of venous thrombosis (hepatic v.; budd chiari) (3) Flushed face due to congestion (4) Itching after bathing (they also have extra mast cells) |
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Treatment of PV?
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Phlebotomy.
Second-line is hydroxyurea. |
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In PV, without treatment, death will usually occur within __________.
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one year
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PV must be distinguished from what?
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Reactive polycythemia
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List the oxygen saturation and EPO findings in PV, lung disease and ectopic EPO production.
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PV: SaO2 normal, EPO is decreased (negative feedback)
Lung disease: SaO2 decreased, EPO increased Ectopic EPO: SaO2 is normal, EPO is high |
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A tumor that typically produces EPO?
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Renal cell carcinoma
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What cells are increased in essential thrombocythemia?
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Especially PLTs. RBCs and granulocytes are also increased.
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Essential thrombocythemia is associated with what mutation?
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JAK2 kinase mutation
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What should you think about when you see tons of platelets in a blood smear?
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Essential thrombocythemia
Iron deficiency anemia (reactive phenomenon) |
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What are the symptoms of essential thrombocythemia?
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Increased risk of bleeding (underfunctioning PLTs) and/or thrombosis (overfunctioning)
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Most myeloproliferative disorders have the ability to ___________, however ET rarely progresses to ___________ or _________.
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burn out; marrow fibrosis; acute leukemia
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In what myeloproliferative disorder is there NOT an increased risk for hyperuricemia and gout?
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ET. No nucleus in PLTs.
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What is myelofibrosis? What mutation is it associated with?
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A myeloproliferative disorder. Neoplastic proliferation of mature myeloid cells, especially megakaryocytes.
Associated with a JAK2 kinase mutation. |
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Why is it called myeloFIBROSIS?
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Because megakaryocytes produce excess PDGF and this results in marrow fibrosis.
We see way too much fibrosis on the picture. Should be fat and bone marrow elements. |
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Clinical features of myelofibrosis include? Explain each feature.
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(1) Splenomegaly due to extramedullary hematopoiesis (fibrosis --> no hematopoiesis in marrow --> hematopoiesis in spleen)
(2) Leukoerythroblastic smear (WBC and RBC immature cells in blood because spleen does not have a reticulin gate preventing their departure from the marrow) (3) Increased risk for infection, thrombosis, bleeding (marrow is gone; spleen is tiny compared to bone marrow, it cannot produce sufficient amounts. |
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What prohibits cell from exciting the marrow when they are too immature?
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reticulin "gates"
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In what myeloproliferative disease do we see these cells?
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Myelofibrosis.
Think of it like this: Some hematopoiesis still occurs in marrow, but it is highly fibrosed and cells get stretched as they try to leave the bone marrow. |