Myeloid Neoplasms, Spleen and Thymus Flashcards

1
Q

Acute Myeloid Leukemia (AML) has an increase in myeloblasts. Describe the steps necessary for this to occur.

A
  • Mutations in hematopoietic stem cells
  • Creation of a leukemic stem cell
  • Proliferates and blocks differentiation
    = accumulation of myeloblasts
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2
Q

What age group is at risk for Acute Myeloid Leukemia?

A

Can occur at ALL ages

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3
Q

Symptoms of Acute Myeloid Leukemia?

A

Fever
Fatigue
Bleeding/bruising

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4
Q

With Acute Myeloid Leukemia, where do the blasts accumulate?

A

BONE MARROW

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5
Q

What defines Acute Myeloid Leukemia?

A

Greater than 20% Blasts in the Bone Marrow

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6
Q

Greater than 20% blasts in the bone marrow?

A

AML (Acute Myeloid Leukemia)

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7
Q

Will there be blasts in the peripheral smear with AML?

A

NO - they are in the bone marrow

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8
Q

What is Aleukemic Leukemia?

A

No blasts in the peripheral smear

ex. AML

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9
Q

What 2 translocations can result in Acute Myeloid Leukemia?

A

t(8;21)

t(15;17)

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10
Q

t(8;21) or t(15;17)

A

Acute Myeloid Leukemia

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11
Q

Which translocation associated with AML involves younger patients with neutrophilic maturation?

A

t(8;21)

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12
Q

What product with the t(8;21) with AML blocks differentiation and causes an accumulation of blasts?

A

RUNX1 - RUNX1T1

- Blocks Core Binding Factor mediated differentiation and maturation

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13
Q

Which translocation associated with AML is known as Acute Promyelocytic Leukemia?

A

t(15;17)

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14
Q

What can be seen in cells with Acute Promyelocytic Leukemia (t(15;17) subtype of AML)?

A

Auer rods

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15
Q

Auer rods are seen with?

A

Acute Promyelocytic Leukemia

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16
Q

Normally, RAR binds ____ and initiates gene transcription for differentiation of myeloid cells

A

RA

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17
Q

With t(15;17) RAR is bound to ____ and cannot bind RA which means?

A

Bound to PML

==> NO differentiation; accumulation of blasts!

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18
Q

What is the treatment for t(15;17) in order for differentiation of the cells to occur?

A

Give All Trans RA

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19
Q

Those with t(15;17) are at a higher risk for?

A

Disseminated Intravascular Coagulation

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20
Q

What are 2 unusual presentations of Acute Myeloid Leukemia?

A

Gingival involvement

Soft tissue mass formation

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21
Q

CLONAL disorder with morphologic manifestations that can range through many cell lineages

A

Myelodysplastic Syndrome

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22
Q

What is considered a precursor to Acute Myeloid Leukemia?

A

Myelodysplastic Syndrome

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23
Q

Myelodysplastic Syndrome is considered a precursor to Acute Myeloid Leukemia. This is especially true if?

A

The Myelodysplastic Syndrome was caused by prior radiation therapy (t-MDS)

24
Q

What do individuals present with, with Myelodysplastic Syndrome?

A

Cytopenias - low blood cells

25
3 classifications for Myelodysplastic Syndrome?
1. Dysmorphic features in 1 or more lineages 2. Chromosomal analysis 3. Increase in BLASTS (but not to level of AML(20%))
26
What type of mutations cause Myelodysplastic Syndrome?
Driver mutations because it is a CLONAL disorder
27
RAEB 1
- seen with Myelodysplastic Syndrome | 5%-9% increase in blasts
28
RAEB 2
- seen with Myelodysplastic Syndrome | 10% - 19% increase in blasts
29
What does RAEB stand for?
Refractory Anemia with Excess Blasts | - Used with Myelodysplastic Syndrome
30
What drives proliferation with Myeloproliferative Neoplasms?
Constitutively active mutated Kinases
31
Describe the overall pathway of what can occur with Myeloproliferative Neoplasms
- Something in bone marrow proliferates - Neoplastic stem cells travel to other sites - Extramedullary Hematopoiesis occurs = Fibrosis and Acute Leukemias occur
32
With Chronic Myeloid Leukemia it is a Myeloproliferative Neoplasm. What cell type proliferates?
Mature myeloid cells
33
What translocation is present with Chronic Myeloid Leukemia?
t(9;22) - BCR-ABL
34
t(9;22) causes a ______ to be constitutively active
Tyrosine kinase
35
What 4 things can classify Chronic Myeloid Leukemia?
Marked Leukocytosis (WBC >100K) Increased Buffy coat Splenomegaly Extramedullary Hematopoiesis
36
What are the 3 phases of CML (Chronic Myeloid Leukemia)?
Chronic phase Accelerated phase Blast phase
37
Blasts increase towards what phase of Chronic Myeloid Leukemia?
Towards the Blast (last) phase
38
Myelofibrosis
Stroma proliferates
39
Polycythemia Vera
RBC proliferates
40
Essential Thrombocythemia
Platelets proliferate
41
What mutations are drivers for growth for myelofibrosis, polycythemia vera, essential thrombocythemia?
JAK2 Kinase mutations
42
Myelofibrosis affects what organ mostly?
Spleen
43
Clonal proliferations (neoplasms) of immature dendritic cells
Langerhans cell Histiocytosis
44
Langerhans cells are what type of cells?
Immature dendritic cells
45
What 2 things can be seen with Langerhans cell Histiocytosis?
1. Grooved nuclei of langerhans cells | 2. Tennis racket shaped Birbeck granules
46
Grooved nuclei of Langerhans cells and Birbeck granules classify?
Langerhans cell Histiocytosis
47
There may also be lots of _____ with Langerhans cell Histiocytosis
Eosinophils
48
What markers are used to identify Langerhans cell Histiocytosis?
S-100 | CD1a
49
What causes reactive splenitis (splenomegaly)?
Viral infection
50
What causes congestive splenomegaly?
Hepatic dysfunction (bc of portal vein)
51
What is Hypersplenism (splenomegaly) associated with?
Cytopenias
52
What usually causes a splenic rupture?
Blunt trauma to abdomen
53
Splenic neoplasms are often involved with ______ malignancies
Hematologic
54
How easy is it for a splenic infarct to occur?
Very easy - blood only has 1 way into the spleen
55
Hyperplasia of the thymus and a Thymoma can both produce?
Autoantibodies
56
If Hyperplasia of the thymus or a Thymoma produce autoantibodies, what results?
Myasthenia gravis
57
Symptoms with Myasthenia Gravis
Muscle weakness that gets worse with activity