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Hematology > Myeloproliferative Neoplasms (Exam III) > Flashcards

Myeloproliferative Neoplasms (Exam III) Flashcards

1
Q

definition of myeloproliferative neoplasm

A

Greoup of disorders that are considered clonal malignancies of the hematopoietic stem cell

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2
Q

Myeloproliferative neoplasm usually affects

A

Usually >40 years old

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3
Q

Myeloproliferative neoplasm

results in

A
  1. BM hyperplasia and Splenomegaly or Hepatosplenomegaly
  2. Dominant expression of one or more “myeloid “ cell lines
  3. Overproduction of one or more of the PB formed elements
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4
Q

Chronic Myeloid Leukemia (CML)

A

granulocytes

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5
Q

Chronic Neutrophilic leukemia

A

neutrophils

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6
Q

Chronic eosinophil (CEL) not otheriwise specified

A

eosinophils

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7
Q

Polycythemia vera (PV)

A

Pancytosis

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8
Q

Essential Thrombocytopenia (ET)

A

Megakaryocytes/PLT

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9
Q

Primary Myelofibrosis (PMF)

A

Fibrosos

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10
Q

Myeloproliferative neoplasm

Transitions

A

Have frequent transitions and many overlapping characteristics between diseases

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11
Q

Chronic Myeloid Leukemia

A

BCR-ABL1+

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12
Q

definition of CML

A

Chronic myeloproliferative disorder with an increase in granulocytes in peripheral blood and marked granulocytic hyperplasia in bone marrow
- PB includs: neutrophils with immature forms, eosinophils and basophils granulocytes

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13
Q

CML also called

A
  • Chronic Myelogenous/ Myelocytic Leukemia
  • Chronic Granulocytic Leukemia
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14
Q

1st human disease traced to a chromosomal abnormality

CML

A

Philadelphia chromosome

  • identified in 90-95% of patients with CML
  • Not diagnostic, also found in 5% of children and 20% of adults with ALL and 2% of patients with AML
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15
Q

CML pathogenesis

A

philadelphia chromosome

  • Chromosome 22q- (shortened chromosome 22)

t(9;22)

increases proliferation and inhibits apoptosis

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16
Q

BCR-ABL+ gene produces

A

p210-tyrosine kinase

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17
Q

p210-kinase causes

A
  1. activation of several signaling pathways
  2. caused increased proliferative capacity
  3. leads to slightly delayed maturation and lacks responsiveness to normal growth regulators
  4. Prevents apoptosis (cell death) in leukemia cells
  5. Considered essential in the pathogenesis of CML
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18
Q

Atypical CML

A

those who don’t have BCR-ABL gene
- Different prognosis and treatment

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19
Q

Not all Philadelphia chrom. or BCR-ABL genes are identical

A
  • fusion protein can result in different sizes, different outcomes and different classification
  • Treatment has to be determined by individual case
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20
Q

CML clinical features

A

Can be asymptomatic or symptomatic
- common to be discovered incidentally

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21
Q

typical CML symptoms

A
  1. Malaise/fatigue = anemia
  2. Abdomen Fullness
  3. Loss of Apetite = splenomegaly
  4. Night sweats
  5. Weight Loss
  6. Bone tenderness/aching
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22
Q

CML phases can be

A

biphasic or triphasic

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23
Q

Chronic CML phase

A
  • Usually diagnosed in this phase
  • Disease remains stable for several years
  • Responsive to chemo
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24
Q

Accelerated phase CML

A

3-5 years after onset (untreated)

Worsening clinical symptoms
- Unexplained fevers
- Significant weight loss
- Worsening Splenomegaly
- Bleeding
- Thrombosis
- Infections

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25
Q

Blast phase CML (Blast cell crisis)

A

Conversion of CML to aggressive form of acute leukemia that is difficult to treat

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26
Q

Chronic CML phase CBC

A

Increased WBC count (<100,000 microL) - Leukocytosis

Decreased Hemoglobin and Hematocrit
- anemia
- Severity is proportional to the degree of leukocytosis

Possible Thrombocytosis (EXAM) TRUE

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27
Q

CML lab findings Chronic Phase

A

Increased WBC - Leukocytosis

Increased Neutrophils with immature forms
- May resemble “shift left”, but not due to BM response to infection
- see all stages from occasional myeloblast (>2%) to segmented neutrophils
- Usually more myelocytes than metamyelocytes –> “Myelocyte Bulge”

Increased Eosinophil and Basophils
- Basophilia - one of the first indicators (>2% in chronic phase)

Possible increased PLT thrombocytosis

Normocytic, Normochromic Anemia (Low RBC)

28
Q

CML Chronic phase

Additional findings

A
  • giant platelets
  • nRBCs
  • Anisocytosis
  • Basophilic stippling
  • polychromasia
29
Q

CML Chronic Phase BM

A

Hypercellular
- marked granulocytic hyperplasia

Blasts not significantly increased (<5%)

Increased Neutrophilic, Basophilic, Eosinophilic cells

Increased Megakaryocytes

30
Q

BM - CML accelerated

A
  • blasts increasing 5-19%
  • Megkaryocyte clusters
  • Fibrosis
31
Q

PB - CML accelerated

A
  • increasing blasts and promyelocytes
  • basophils more or equal to 20%
  • Thrombocytopenia

Worsening splenomegaly, bone pain, anemia

32
Q

CML blast phase

BM

A
  • Blasts, more or equal to 20% and in clusters
  • increased fibrosis
  • dysplastic features
33
Q

CML blast phase

PB

A

Blasts >20%

conversion to Acute Leukemia

34
Q

Cytogenetics CML

A

Detection of t(9;22)(q34;q11)(BCR-ABL+)

performed by:
- FISH = Fluorescnece in Situ Hybridization
- RT-PCR = reverse transcriptase polymerase chain reaction

35
Q

RT-PCR

A
  • extremely sensitive
  • Used to detect residual disease or recurrence after treatment
36
Q

CML - LAP

A

LOW LAP score

Stains leukoyte alkaline phosphatase activity in granules
- present in normal neutrophilic granules
- decreased/absent in malignant neutrophilic granules
-

Returns to normal or increased due to
- remission of disease with therapy
- infection

37
Q

Toxic vacuoles

A

Leukomoid RXN: Increased

CML: Absent

38
Q

Toxic granulation

A

Leukomoid RXN: Increased

CML: Absent

39
Q

Dohle Bodies

A

Leukomoid RXN: Increased

CML: Absent

40
Q

Eosinophilia

A

Leukomoid RXN: Normal

CML: Increased

41
Q

Basophilia

A

Leukomoid RXN: normal

CML: increased

42
Q

Myelocyte bulge

A

Leukomoid RXN: absent

CML: present

43
Q

LAP score

A

Leukomoid RXN: high

CML: low

44
Q

Ph Chromosome

A

Leukomoid RXN: negative

CML: positive

45
Q

CML prognosis

A

Medial survival 4-6 years

Poor prognostic indicators predicting blast transformation
- Additional chromosomes abnormalities
- Severe hepatosplenomegaky
- Change in PLT count - Thrombocytosis to Thrombocytopenia
- Extreme leukocytosis >100,000 uL
- BM blasts >5%
- PB basophils >20%
- Tumor suppressor gene p53 - may induce drug resistance

46
Q

CML treatment

A

Myelosuppressive treatments (cytotoxic therapy)

Tyrosine Kinase Inhibitor

Allogenic bone marrow transplant

47
Q

Treatment for CML

Myelosuppressive treatments (Cytotoxic therapy)

A
  • hydroxyurea, interferon-alpha, or busulfan tehrapy

Targeted to controlling hyperproliferation of myeloid elements by inhibiting cell division

48
Q

Allogenic bone marrow transplant

CML treatment

A

ONLY PROVEN CURE

Transplant related mortality and Graft vs. Host disease
- worse in older patients
- have to consider risks associated

49
Q

Tyrosine kinase inhibitor

CML treatment

A

Imatinib mesylate (Gleevec)
- inhibits proliferation, slows skin cell growht, and induces cell death
- first drug developed against a specific molecular target
- Treats all phases of CML

50
Q

Chronic Neutrophilic Leukemia (CNL)

PB

A

major criteria: WBC count >25,000/uL

and

> 80% band or segmented neutrophils

51
Q

CNL most cases

A

mutation CSF3R

52
Q

CNL characteristics

A

Basophilia and Eosinophilia NOT present

Does NOT have philadelphia chromosome

LAP increased

53
Q

Chronic Eosinophilic leukemia (CEL)

A

clonal expansion of eosinophil precusors

54
Q

major criteria CEL

A

PB eosinophils >1,500 microL

(normal range: 0-500 microL)

Does not have Ph Chromosome

Tissue damage occurs from eosinophilic granules

55
Q

Polycythemia Vera Definition (PV)

A

Chronic abnormaility of the hematopoietic stem cell characterized by uncontrolled proliferation of
- erythroid cells
- granulocytic cells (neutrophils)
- Megakaryocytic cells

56
Q

Etiology PV

A

current theory:

JAK2 V617F mutation, allowing for RBC production independent of erythropoietin (found in 90-95% cases)

57
Q

PV clinical features

A

Chronic disease with insidious onset (asymptomatic/routine doctor visit)

possible symptoms
- Thrombosis/bleeding
- Splenomegaly and/or hepatomegaly
- hyper viscosity
- Hypertension
- Gout

58
Q

PV CBC + Peripheral smear

A

Pancytosis
- Increased RBC count, Hgb, Hct
- Increased PLT count (thrombocytosis) - abnormal morphology/fxn
- Increased relative and absolute Granulocytosis - mostly enutrophils, but can see increase in Eosinophil and Basophils

59
Q

PV - BM findings

A

Hypercellularity with Pancytosis
- erythroid, Myeloid, Megakaryoctic Hyperplasia

60
Q

Secondary Polycythemia

A

Increased production of RBCs due to response to other factors
- hypoxia
- Erythropoeietin (EPO) producing tumor

61
Q

Relative Polycythemia

A

Increased RBC count due to decreased plasma volume
- dehydration/burns

AKA tertiary polycythemia

62
Q

Differential diagnosis

Secondary polycythemia

A

secondary: RBCs only
PV: multiple cell lines

63
Q

EPO
(secondary vs PV)

A

Secondary: increased

PV: decreased

64
Q

Relative vs PV increased cells

A

Relative: RBCs only

PV: multiple lines

65
Q

PV treatment

A

Therapeutic phlebotomy
- want to create a frequency (1 unit/weekly) to bring hematocrit below:
1. men <45%
2. Women <40%

other options:
- Myelosuprressive therapy
- radioactive phosphoruous (UV radiation absorbed into the BM)

66
Q

Essentiak Thrombocythemia (ET) - definition

A

Chronic myeloproliferative disorder characteristics by marked thrombocytosis associated with abnormal platelet function and an increased risk of thrombosis and hemorrhage

67
Q

Common mutations in ET

A

Hematology (36 decks)

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