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Hematology II > Myeloproliferative Neoplasms (MPNs) > Flashcards

Myeloproliferative Neoplasms (MPNs) Flashcards

1
Q

Define myeloproliferative neoplasm

A

Clonal disorder of HSC caused by genetic mutations. This leads to reduced negative feedback, thus malignant overproliferation of cells in BM, blood, and other tissues

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2
Q

What are the two main classifications of leukemias?

A
  1. Acute: acute nonlymphocytic leukemias (ANLLs)
  2. Chronic: Myeloproliferative neoplasms
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3
Q

List diseases included the MPN category

A
  • Chronic myeloid (or myelocytic) leukemia (CML)
  • Chronic neutrophilic leukemia
  • Polycythemia
  • Chronic myelomonocytic leukemia
  • Essential thrombocytopenia
  • Primary myelofibrosis
  • Chronic eosinophilic leukemia, not otherwise specified
  • MPN, unclassifiable
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4
Q

List common traits of chronic MPNs

A
  • Panhyperplasia of BM
  • Extramedullary hematopoiesis
  • BM fibrosis
  • Overlapping manifestations btwn diseases
  • Frequently end in acute leukemia
  • Increased megakaryocytes in BM
  • Platelet dysfunction
  • Cytogenetic abnormalities
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5
Q

Traits of CML?

A
  • Most common over 45 y/o
  • Abnormal Philadelphia chromosome
  • Rare eosinophilic and basophilic leukemia -> very acute
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6
Q

What is the Philadelphia chromosome (Ph22) abnormality?

A

Translocation (gene swap) between two chromosomes such that tyrosine kinase activity is increased. This leads to uncontrolled production of abnormal blood cells. Apoptosis lost

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7
Q

What are CML lab findings (there’s a lot, sorry!)?

A
  • Left shift (blasts < 10%) bc Ph22 causes premature cells to be released)
  • WBC 50,000-800,000/mm3 (blood resembles BM)
  • ~90% are Ph22 positive
  • Ph22 negative CML tend to have atypical disease, more acute, less responsive to chemo
  • Reduced LAP
  • Usually remarkable thrombocytosis
  • Anemia (may see nRBCs)
  • Increased basophils
  • Hypercelullar BM, M:E is 10:1 to 50:1
  • May see pseudo-Gaucher cells
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8
Q

List lab findings of CML-AP (accelerated phase)

A
  • Blasts range 10%-19% in blood and/or BM
  • Blood basophils >= 20%
  • High WBC unresponsive to treatment
  • Super high PLT unresponsive to treatment
  • Very low PLT also possible, not caused by treatment
  • Clonal cytogenetic abnormality
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9
Q

List lab findings for CML-BP (blast phase or blast crisis)

A
  • Blasts >= 20% in blood and/or BM
  • Transforms to AML (M2 usually) or ALL (lymphoblasts > 5%) or rarely others
  • Myeloid sarcoma (**ICC: idk what this is someone please correct this info)
  • 2-6 month life expectancy
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10
Q

How do you treat CML?

A
  • BM transplant
  • OHSU doc came up with drug to inhibit mutant tyrosine kinase -> prolongs chronic phase but blast crisis still happens
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11
Q

List lab findings for CNL (neutrophilic) with OR without CSF3R mutation

A
  • Rare, avg 1.8 yr survival rate
  • PMNs or bands >= 80% (no left shift)
  • Hypercellular BM (M:E may be greater than 20:1)
  • Neutrophilic leukocytosis persists without left shift evidence and absence of sepsis
  • Toxic granules and Dohle bodies
  • High LAP (350-400)
  • Slightly reduced platelet
  • Mild anemia
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12
Q

Differences between leukemoid reaction and CNL in terms of LAP score and left shift?

A

Leukemoid reaction: High LAP and left shift evident

CNL: High LAP but NO left shift apparent

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13
Q

Describe chronic monocytic leukemia

A
  • New classification is chronic myelomonocytic leukemia (CMML)
  • Myelodysplastic and MPN (see overlap between the two)
  • If doesn’t meet criteria of CMML -> ID as clonal monocytosis of undetermined significance
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14
Q

Define polycythemia

A

Malignant increase in red cell mass (better indicator of how many RBCs there are) characterized by JAK2 mutation

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15
Q

List polycythemias

A
  • Polycythemia vera/rubra vera
  • Secondary polycythemia
  • Relative erythrocytosis
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16
Q

List lab findings of polycythemia vera

A
  • Hgb and/or Hct increase
    - M Hgb > 16.5 g/dL, Hct > 49%
    - F Hgb > 16.0 g/dL, Hct > 48 %
  • Initially, RBC’s look normal
  • Hypercellular BM due to malignancies (too much erythropoiesis, myelopoesis, and megakaryopoiesis)
  • Ineffective extramedullary hematopoiesis leads to increased aniso and poik as disease progresses
17
Q

Describe JAK2 mutation

A
  • Leads to endogenous erythroid colony formation (EEC), which means the body doesn’t need EPO to produce RBCs
  • Body responds to mutation effects by decreasing EPO in attempt to shut down RBC production. This doesn’t matter tho bc malignant RBCs don’t need EPO to be made anyway
18
Q

List more lab findings of polycythemia vera (didn’t want to put them all on same flash card cuz too clunky). Hint: about LAP and O2 saturation

A
  • Increased granulocytes and platelets, so see high LAP
  • Normal arterial O2 saturation
  • Careful when drawing blue top tube bc increased RBC mass -> reduced plasma volume, which means too much sodium citrate in tube. Must do calc to remove anticoag (we’re not doing this yet)
  • Hemostasis results may be affected by wrong anticoagulant to blood plasma ratio
19
Q

Describe iron deficiency characteristics in polycythemia vera

A
  • PLT hypofunction -> keep bleeding
  • Repeated therapeutic phlebotomy (NOT lobotomy…I ‘m so tired I misread it that way)
  • Increased RBC production and Fe turnover
  • Reduced RBC survival (this is good bc limits expanding RBC mass. More RBC mass increases chance of thrombosis)
20
Q

Describe RBC morphology in polycythemia vera

A
  • Long-term PV leads to tear drop cells due to increasing myelofibrosis
  • Become anemic bc body gets tired of putting in effort to make RBCS, so it just gives up. This is called spent phase aka post polycythemia myeloid metaplasia
21
Q

Anemia with leukoerythroblastosis (immature RBC And WBC seen in peripheral blood) is seen in which disease state?

A

Myeloid metaplasia

22
Q

What causes secondary polycythemia? Lab findings?

A
  • Cause: Hypoxia (so it’s not malignant)
  • Labs: Reduced arterial O2 saturation, increased EPO, Increased RBC mass due to more BM erythropoiesis, normal granulocytes and PLTS so normal LAP
23
Q

What are the two most common groups that feature relative erythrocytosis?

A
  1. Dehydrated groups most common
  2. Asymptomatic middle-aged white males that are hypertensive, obese, and have long smoking history
24
Q

Lab findings in relative erythrocytosis?

A
  • Reduced plasma volume (leads to elevated Hct, but normal RBC mass due to reduced plasma volume)
  • Normal O2 saturation
  • Normal EPO, it just falsely appears like increased RBC production
  • Normal LAP
25
Q

Lab findings for essential thrombocytopenia (ET)?

A
  • Increased BM megakaryopoiesis and slightly increased granulopoiesis
  • PLT > 600,000/mm3
  • Spontaneous PLT aggregation and function defects (thrombotic + hemorrhage complications)
  • JAK2 gene mutation + EEC in 30-50% of pts
26
Q

Describe what you see in blood smear in patients affected with ET

A
  • Large masses of PLT aggregates
  • Giant, bizarre PLTs
  • Occ. megakaryocyte fragment
  • Mild normocytic, normochromic anemia, unless Fe deficiency due to too much bleeding
  • Neutrophilia, occ increased eos and basos
27
Q

Describe WBC, PLT, and RBC findings in early primary myelofibrosis (PMF)

A
  • WBCs and PLTs may be increased, normal, or decreased
  • May see increased basos + eos
  • Immature granulocytes + pseudo Pelger-Huet
  • Micromegakaryocytes
  • May have abnormal PLT shape and impaired function (leads to too much bleeding)
  • RBCs: anemia with nRBCs, polychromatic, and bizarre shapes
  • Hallmark of disease = teardrops
28
Q

Describe general presentation of primary myelofibrosis (PMF)

A
  • Can present de novo or progression of PV or ET
  • Most have JAK2, CALR, or MPL mutation -> higher CD34+ count -> correlates to progression to marrow fibrosis
  • Clonal cells stimulate release of fibroblastic growth factor
  • BM dry tap common so do biopsy for best luck
29
Q

Describe PMF presentation once disease progresses to more serious form

A
  • Ineffective erythropoiesis + hemolysis
  • Extramedullary hematopoiesis, so see more nRBCs and poik
  • Reduced WBC and PLT (see immature granulocytes, megakaryocytic fragments, and microplatelets)
30
Q

Describe Chronic eosinophilic leukemia not otherwise specified (CEL NOS)

A
  • Eosinophilia with no signs of allergy, parasitic infections, or other malignancies associated with this
  • Myeloblasts: may be elevated in BM but less than 20%
  • Eosinophils infiltrate tissues and organs leading to organ dysfunction
31
Q

List other MPNs

A
  • iHES (idiopathic hypereosinophilic syndrome)
  • M/LN-eo (myeloid lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions
  • Systemic mastocytosis
  • MPN-U (MPN, unclassifiable)

Hematology II (8 decks)

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