M. Acute Leukemia (L15) Flashcards

1
Q

What percentage of bone marrow cells are blasts normally? What percentage is seen in Acute Leukemia?

A
  • < 3%

* >20%

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2
Q

What is the general pathophysiology of Acute Leukemia?

A
  • Disruption of hematopoiesis –> inability for cells to mature –> buildup of myloid or lymphoid blasts/stem cells
  • Proliferative cells crowd out normal cells in bone marrow –> acute presentation of anemia, thrombocytopenia & neutropenia
  • Blasts enter blood –> increased (lymphocytic or myelocytic) WBC in peripheral blood (large immature cells with little cytoplasm and nucleolus)
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3
Q

What “step” in hematopoiesis are acute leukemias “stuck” in?

A

• Transition from HSC to lymphoblast and myloblast but then get stuck

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4
Q

What are the 2 main types of Acute Leukemia & what type of proliferative cell type is present? Which is most common? Mnemonic?

A

• Acute Myloid Leukemia (AML) = myloblasts
• Acute Lymphoid Leukemia (ALL) = Lymphoblasts
• AML is most common
M for More (L for little)

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5
Q

How does Acute Leukemia present on Bone Marrow Biopsy?

A

• 100% cellularity (no fat cells)

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6
Q

What is the “rule of thumb” to estimate what percentage of bone marrow should normally be cellular?

A

100 – age

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7
Q

What age groups are AML & ALL found in? Mnemonic?

A
  • ALL = children (Think L for little)

* AML = adults (M = more for more common)

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8
Q

Time wise, what is the onset of Acute Leukemia?

A
  • Presents with severe symptoms within a few weeks

* Can rapidly become fatal if not treated

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9
Q

What is the clinical presentation for Acute Leukemia?

A
  • Crowding in BM by proliferative cells –> Pancytopenia –> below symptoms:
  • Anemia –> fatigue & pallor
  • Thrombocytopenia –> bleeds/bruising
  • Decrease in functional WBCs –> infection & fever
  • Leukocytosis as proliferative WBCs spill into circulation
  • Hepatosplenomegaly
  • Skin and gum WBC infiltration
  • CNS involvement
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10
Q

What is the leading cause of non-traumatic death in children?

A

ALL

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11
Q

What ethnic group and sex is ALL most common in?

A

White boys

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12
Q

What increases the likelihood of ALL?

A
  • Down syndrome
  • Radiation
  • Genetic syndromes
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13
Q

What are the 2 main types of ALL? Which is more common? Which is more aggressive?

A
  • Pre-T Cell ALL
  • Pre-B Cell ALL
  • B is more common (85%)
  • T is more aggressive
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14
Q

Where do Pre-T ALL masses present most often?

A
  • Superior Mediastinum (where thymus is)

* Cause SOB as compress structures

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15
Q

What is the key finding for the L3 FAB classification on bone marow aspirate smear? What disease is it related with?

A
  • Vacuoles

* Burkitts

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16
Q

What are characteristics that lead to a good prognosis for ALL? Bad?

A
  • 50k bad)
  • 1-10 yo (10 bad)
  • Lack of CNS involvement (CNS involvement bad)
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17
Q

What is the overall prognosis of ALL?

A
  • Good, 98% go into remission

* 2/3 have long term remission while 1/3 relapse

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18
Q

What age group is de novo AL seen in? What are the cytogenetic characteristics? Better or worse prognosis?

A
  • Younger age children
  • Less complex cytogenetics
  • Better prognosis
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19
Q

What causes secondary Leukemia?

A
  • Prior diagnosis of myeloproliferative disorder or myelodysplastic disorder
  • Toxins (occupational, chemo, accidental)
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20
Q

What are Auer Rods diagnostic of?

A

AML

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21
Q

What is the overall prognosis of AML?

A
  • 60% remission

* 80% of remission has reoccurrence

22
Q

What is butterfly shaped nuclei diagnostic of?

A

• APL, a type of AML

23
Q

How is the prognosis of ALL & AML changed if t(9:22) translocation is present?

A

Worse Prognosis

24
Q

What mutation causes Acute Promyelocyte Leukemia (APL)?

A

• T(15:17) PML:RARalpha
• Fuses anti-apoptotic factor & retinoic acid receptor –> blocks myeloid proliferation
DIC PRONE

25
Q

DIC is seen in what type of Acute Leukemia?

A

APL

26
Q

What type of Acute Leukemia is one of the first examples of targeted gene therapy?

A

APL (AML)

27
Q

What type of AL stains with myeloperoxidase (MPO)? Color of MPO?

A

AML

Dark blue

28
Q

What is the main morphological feature of lymphoblasts and myloblasts?

A

• Large size which is mostly made up of nucleus

29
Q

None specific esterase stains what cell type? Color of stain?

A

Megaloblasts

Greenish red color

30
Q

What is a neoplastic buildup of lymphocytes called? How about mylocytes?

A

ALL

AML

31
Q

How does incidence for AML & ALL change in relationship to age?

A
ALL = bimodal but much higher in kids than in elderly
AML = risk increases with age
32
Q

What does M3 in the FAB classification system signify?

A

APL

33
Q

Does acute leukemia present with peripheral leukocytosis or leukopenia?

A

Either

There is a decrease in functional WBCs, but the proliferative WBCs may or may not spill into the circulation

34
Q

Is extramedullary involvement more often seen in AML or ALL?

A

ALL

35
Q

What condition can be a precursor to ALL?

A

Initially present as nodal or skin disorder –> progress to bone marrow

36
Q

Most common cancer in newborn to 15yo? 15-40? 40-60? >60?

A

ALL
AML
AML (60%) or CML (40%)
CLL

37
Q

4 types of AML based on lineage?

A

Acute Monocytic Leukemia
Acute Erythroid Leukemia
Acute Megacaryocytic Leukemia
Acute Promyelocytic Leukemia (APL)

38
Q

What type of acute leukemia is associated with DIC?

A

APL (type of AML)

39
Q

Patients with what type of AML are prone to bleeding?

A

APL because of the associated DIC

40
Q

For what type of Acute Leukemia is all trans retinoic acid (Vit A) a treatment?

A

APL

41
Q

What cytogenetic conditions are associated with a better ALL prognosis?

A

t(12:21)

Hyperploidy (Trisomy 4 & 10)

42
Q

What cytogenetic conditions are associated with a worse ALL prognosis?

A

t(9:22)
t(1:19)
MLL gene

43
Q

What is the L1 category of ALL?

A

Lymphoblasts with very little cytoplasm (very high N/c ratio)

44
Q

What types of extramedullary involvement is seen in pre-T & pre-B ALL?

A

Both = CNS, Skin, Liver Spleen

B only = Gonads, Lymph nodes

45
Q

Are neoplasms of precursors more common in children or adults? What about effector cell neoplasms?

A

Neoplasms of precursor cells tend to be more common in children, while those of antigen-dependent effector and memory cells tend to be more common in adults.

46
Q

Where do different types of neoplastic cells go?

A

–neoplasms of bone marrow precursor cells are often acute leukemias;
–those of germinal center cells occur in follicular areas of lymph nodes and other tissues,
–those of memory B-cells are often found in sites of antigenic stimulation, such as the gastrointestinal tract.

47
Q

What type of cells are building up in chronic leukemia?

A

Abnormal but relatively mature cells

48
Q

How urgently must treatment be started for acute leukemias? Chronic?

A
Acute = begin immediately
Chronic = sometimes observe for a while before begin
49
Q

What is taken into account for FAB & WHO classifications?

A
FAB = morphology and cytochemistry
WHO = morphology, cytochemistry & clinical presentation
50
Q

What are Auer rods made of?

A

Fusion of primary granules