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Hematology > Hematology Week 3: Chronic Myeloid Leukemia > Flashcards

Hematology Week 3: Chronic Myeloid Leukemia Flashcards

1
Q

Case 1

A
  • not acutely ill but borderline sick
  • dx is often incidental, patients might not be that sick but can be an incidental finding
  • Fatigue is a very common presentation
  • Weight loss = splenomegaly pushing on the belly
  • so early Satiety

Chronic Myeloid Leukemia

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2
Q

CML Blood Smear

3 listed

A
  • WBC extremely high
  • Abs Basophil count is very high
  • very significant left shift
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3
Q

If only myeloblasts are proliferating?

A

Acute Myeloid Leukemia (AML)

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4
Q

If all myeloid lineages are proliferating?

A

Chronic Myeloid Leukemia (CML)

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5
Q

Immature Myeloid

A

Acute Myeloid Leukemia (AML)

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6
Q

Immature Lymphoid AKA

A

Acute Lymphoblastic Leukemia (ALL)

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7
Q

Mature Myeloid AKA

A

Chronic Myeloid Leukemia (CML)

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8
Q

Mature Lymphoid AKA

A

Chronic Lymphocytic Leukemia (CLL)

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9
Q

CML Phases

3 listed

A
  • Chronic Phase
  • Accelerated Phase
  • Blast Phase
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10
Q

CML Accelerated Phase Diagnostic Criteria

7 listed

A
  • 10-19% blasts in periphery or BM
  • OR >20% basophils in periphery
  • OR persistent thrombocytopenia (not treatment-related)
  • OR Persistent thrombocytosis
  • OR increasing WBC
  • OR increasing splenomegaly
  • OR all responsive to therapy
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11
Q

CML Blast Phase Diagnostic Criteria

3 Listed

A
  • >20% blasts in the periphery or BM
  • Clusters of blasts in BM
  • Extramedullary blasts
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12
Q

CML Chronic Phase Diagnostic Criteria

2 listed

A
  • <10% blasts in the periphery or BM
  • often dx is incidental
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13
Q

Most CML is diagnosed in which phase?

A

chronic phase of CML

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14
Q

Without treatment chronic phase CML will progress to _____ in _____ years and transform into?

A
  • Accelerated phase/Blast Phase in 3-5 years
  • AML or ALL
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15
Q

Benign Leukemoid reaction blood smear shows

4 listed

A
  • Increased WBC count <50x109
  • mild left shift
  • No basophilia
  • Toxic changes are present (indicating response to infectious or inflammatory process)
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16
Q

% of cells in blood

A
  • Leukemoid reaction - still neutrophil and band predominance, some metamyelocytes, few myelocytes and blasts are rare
  • CML - the predominance of bands and neutrophils but there are more immature forms and a myelocyte bulge (have over predominance of myelocytes)
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17
Q

LAP Score

3 listed

A
  • Historical test for boards
  • Leukocyte alkaline phosphatase (LAP) Activity can be visualized in granulocytes by a special stain
  • Indicates activation of neutrophils to fight off infection or inflammatory process
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18
Q

High overall LAP Score indicates?

A

activated neutrophils as seen in reactive neutrophilia

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19
Q

Low overall LAP Score indicates?

A

non-activated neutrophils more typical of CML

20
Q

CML Vs Leukemoid Reaction

6 listed

A
  • Leukemoid reaction has toxic changes seen by LAP score
  • CML shows basophilia
  • CML shows Myelocyte bulge pattern
  • Leukemoid reaction shows clinical evidence of infection
  • CML shows very high WBC count
  • CML shows low LAP score
21
Q

Key Genetic Finding in CML

3 listed

A

The Philadelphia Chromosome

t(9;22)(q34;q11.2)

BCR-ABL1 gene fusion

22
Q

Philadelphia chromosome is tested for with?

A

FISH Test

23
Q

The most common mutation associated with Myeloproliferative Neoplasms

A

Tyrosine Kinase Mutations

24
Q

Abnormal BCR-ABL1 fusion protein

A

Overactive tyrosine kinase

25
Q

Imatinib (Gleevec) MOA

A
  • Block Overactive Tyrosine Kinase
  • inhibits BCR-ABL1 protein and block activity
26
Q

CML Treatment Prior to Imatinib

A

interferon/HSCT - high toxicity morbidity/mortality

27
Q

Examples of other Tyrosine Kinase Inhibitors

5 listed

A

Other TKIs

  • Dasatinib
  • Nilotinib
  • Bosutinib
  • Ponatinib
  • Imatinib
28
Q

Imatinib Toxicities

3 listed

A
  • Fluid retention
  • Muscle pain/cramps
  • GI disturbance
29
Q

Dasatinib Toxicities

2 listed

A
  • 20-25% pleural effusions
  • rare pulmonary arterial hypertension
30
Q

Nilotinib Toxicities

5 listed

A
  • Vascular events
  • peripheral arterial occlusive disease
  • CAD
  • Hyperglycemia
  • hypercholesterolemia
31
Q

Imatinib Complete Hematologic Response

5 listed

A
  • normalizes CBC
  • WBC <10
  • Plts < 450
  • no left shift
  • disappearance of splenomegaly
32
Q

Imatinib Cytogenetic Response

2 listed

A
  • No Ph+ metaphases
  • when karyotype is done t(9;22) won’t be found
33
Q

Imatinib Molecular Response

2 listed

A
  • complete - no detectable BCR-ABL mRNA by PCR
  • Major -> 3 log reduction in BCR-ABL mRNA from baseline
34
Q

Imatinib Relapse

A
  • any loss of hematologic or cytogenetic response
  • First check compliance
35
Q

What to do if Imatinib relapse?

A

First check for compliance

36
Q

1 reason for relapse of CML

A

Medication non-compliance

37
Q

PCR detection of BCR-ABL1 fusion detection

A
38
Q

Tests for BCR-ABL1

3 listed

A
  • Karyotype to visualize translocation
  • FISH
  • PCR for BCR-ABL1
39
Q

PCR BCR-ABL1 Transcripts

A
40
Q

1000x Reduction in BCR-ABL1

A

Major molecular response

41
Q

3 log Reduction in BCR-ABL1

A

Major Molecular Response

42
Q

Question 1

A

D. It is associated with CML and CML is associated with basophilia

Leukemoid reaction is not associated with basophilia

43
Q

Question 2

A

B No

  • CML cannot be cured as of yet but BCR-ABL1 can be inhibited by Imatinib
  • the cancerous cells aren’t eradicated but are prevented from proliferating
44
Q

Question 3

A
  • check for compliance
  • however additional mutations can occur and TKI wont be able to inhibit now
  • solution is switch to a different TKI
45
Q

Nomenclature

A
  • overlap in naming CML
  • can be named a myeloproliferative neoplasm and a big 4 leukemia
46
Q

Key Questions to answer

A

Hematology (26 decks)

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