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CMBM Exam 5 > Myeloproliferative Neoplasms and Myelodysplasia > Flashcards

Myeloproliferative Neoplasms and Myelodysplasia Flashcards

1
Q

Common Myeloproliferative Neoplasms

A
  • Chronic melogenous leukemia (CML)
  • Polycythemia vera
  • Primary myelofibrosis
  • Essential thrombocythemia
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2
Q

Myeloproliferative Neoplasms General Symptoms

A
  • Usually increased bone marrow cellularity
  • Slightly increased marrow blasts (<10%)
  • Normal morphology
  • Effective hematopoiesis
  • CBC- increased in one or more cell lines
  • Organomegaly common; such as enlarged spleen
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3
Q

Tyrosine Kinases

A
  • Enzyme that transfers phosphate group from ATP to a protein in a cell
  • On/off switch for cellular function
  • Disruption in the genes controlling these products lead to dysregulated/uncontrolled proliferation
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4
Q

Myeloproliferative Neoplasms Chart

A
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5
Q

Chronic Myelogenous Leukemia (CML)

A
  • Prototypical myeloproliferative disorder
  • Neutrophils w/ varying stages of maturation and increased basophils, hypercellular bone marrow w/ maturation of all cell lines; very little adipose tissue present in bone marrow as a result
  • Most pts in 50-60’s
  • Translocation b/w chromosome 9 to 22 (Philadelphia chromosome) = BCR-ABL fusion gene
  • Most pts. diagnosed in chronic phase where white count is high but relatively stable
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6
Q

CML Chronic Phase

A
  • Leukocytosis- median WBC count 100x 10^9/L (N is <10.8x 10^9/L), mainly neutrophils lineage
  • Blasts usually <2% of WBCs
  • Megakaryocytes small and hypolobated
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7
Q

CML Accelerated Phase Criteria

A

Any 1 or more of the following hematologic/cytogenetic criteria or response-to-TKI criteria:

  • Persistent or increasing WBC (>10 x 10 ^9/L), unresponsive to therapy
  • Persistent orincreasing splenomegaly, unresponsive to therapy
  • Persistent thrombocytosis (>1000 x 10^9/L), unresponsive to therapy
  • 10-19% blasts in the PB and/or BM
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8
Q

CML Blast Phase

A
  • Evolving into acute leukemia (usually AML but can be ALL)

*blasts >20% of peripheral blood WBC or BM

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9
Q

CML Therapy

A
  • Gleevec

*management of malignant clone; keep normal myeloid elements at sustainable level with reduced cytogenetic expression of fusion gene

*not a cure; can only work for so long

  • Bone marrow transplant

*only potential curative option

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10
Q

Polycythemia Vera

A
  • Increased RBC production independent of normal erythropoiesis
  • Almost all have JAK2 mutation or variant thereof
  • Increased red cell mass and panmyelosis
  • Median survival is 13 years
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11
Q

Polycythemia Vera (PV) Symptoms

A
  • Related to hypertension/vascular abnormalities due to increased red cell mass

*venous or arterial thrombosis

*myocardial infarction or stroke

*portal-splenic vein thrombosis/Budd-Chiari syndrome

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12
Q

Jak-2 Mutation

A
  • Mutation is specific to myeloid neoplasms and confers proliferation advantage
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13
Q

Polycythemia Vera Diagnostic Criteria

A
  • MAJOR:

*Hgb >16.5 g/dL for men; >16 g/dL for women (normal for men is ~13.5-17.0 g/dL, women 12.0 to 15.0 g/dL)

*presence of JAK2 mutation (no BCR-ABL1)

*no cause of secondary erythropoiesis (elevation of erythropoietin (EPO) due to hypoxia, high oxygen affinity Hb, EPO production by tumor)

  • MINOR:

*BM hypercellularity w/ trilineage growth

*serum erythropoietin below reference range

*endogenous colony formation in vitro

  • WHO PV criteria

*Hemoglobin >16.5 g/dL in men, >16.0 g/dL in women

*BM biopsy show hypercellularity for age w/ trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation w/ pleomorphic, mature megakaryocytes (diff. in size)

*presence of JAK2V617F or JAK2 exon 12 mutation

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14
Q

Polycythemia Vera 3 Phases

A
  • Prodromal, prepolycythemic- only mild erythrocytosis
  • Overt polycythemic- significantly increased RBC mass
  • Spent phase- myeloproliferative and pancytopenia; extensive marrow fibrosis in spent phase (yellow/black stain for reticular fibers)
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15
Q

Budd-Chiari Syndrome

A
  • PV is most common cause
  • Thrombotic or non-thrombotic obstruction to hepatic venous outflow
  • Hepatomegaly, ascites and abdominal pain = high mortalit (blood is able to get into liver but is hard to get out)
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16
Q

Primary Myelofibrosis (PMF)

A
  • Proliferation of megakaryocytes w/ reticulin and collagen fibrosis
  • Does not meet criteria for CML, PV, MDS or other
  • Fibrotic marrow causes RBCs to become pear shaped, and leukoerythoblastosis = immature cells being forced into blood
17
Q

Essential Thrombocythemia (ET)

A
  • Platelet count >450 x 10^9/L
  • Megakaryocytes proliferation w/ large and mature morphology
  • HUGE, hyperlobated megakaryocytes easily distinguished from micro-megakaryocytes of CML
  • Doesn’t meet criteria for CML, PV, PMF, MDS or other myeloid neoplasm
  • Splenic or hepatic vein thrombosis
18
Q

Elevated Tryptase Seen In

A
  • Anaphylaxis and mastocytosis
19
Q

Myelodysplasia/Myeloproliferative Disease

A
  • Chronic myelomonocytic leukemia (CMML); not a ture leukemia
  • Disease of older adults, incidence unknown
  • Persistent peripheral blood monocytosis >1 x 10^9/L
  • No Philadelphia chromosome
20
Q

CMML to AML

A
  • >20% blasts in blood/marrow
21
Q

Juvenile Myelomonocytic Leukemia (JMML)

A
  • Childhood version of CML
  • Proliferation of granulocytic and monocytic lineages
  • 10% of cases ass’d w/ neurofibromatosis type 1
  • Diagnostic criteria: no Philadelphia chromosome
22
Q

Neurofibromatosis-1

A
  • 200-500-fold increased risk of developing myeloid malignancy
23
Q

Myelodysplasia

A
  • Group of clonal hematopoietic stem/multipotent cell disease
  • Dysplasia and ineffective hematopoiesis of one or more myeloid cell lines
  • can evolve into AML
  • Usually increased bone marrow cellularity
  • CBC w/ cytopenias; too few cell lineages
24
Q

Ringed Sideroblasts

A
  • Majority of iron entering RBCs is incorporated into heme
  • Rest is stored as ferritin
  • Always pathologic sign
25
Q

Myelodysplasia Syndromes 1

A
26
Q

Refractory Anemia

A
  • Dysplasia only in erythroid line
  • Progression to acute leukemia low (6%)
27
Q

Refractory Anemia w/ Ringed Sideroblasts

A
  • Dysplasia only in erythroid line
  • Progression to acute leukemia is low (1%)
28
Q

Refractory Cytopenia w/ Multilineage Dysplasia

A
  • Dysplasia in 2+ myeloid cell lines
  • Accounts for 24% of MDS cases
29
Q

Myelodysplasia Syndromes 2

A
30
Q

Myelodysplasia Syndromes 3

A
31
Q

Refractory Anemia w/ Excess Blasts (RAEB)

A
  • RAEB-1: 5-9% blasts in marrow
  • RAEB-2: 10-19% blasts in marrow
  • Progression to acute leukemia 25-35% (greatest likelihood of all MDS)
32
Q

Myelodysplasia w/ 5q-

A
  • Defined by deletion 5q
  • Disease of middle aged to elderly women
  • Long survival
  • Progression to acute leukemia rare
33
Q

Treatment of MDS

A
  • Supportive care
  • Red cell transfusions

CMBM Exam 5 (11 decks)

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