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SBL > Myeloid Neoplasms, Spleen and Thymus > Flashcards

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
Q

3 classifications for Myelodysplastic Syndrome?

A
  1. Dysmorphic features in 1 or more lineages
  2. Chromosomal analysis
  3. Increase in BLASTS (but not to level of AML(20%))
26
Q

What type of mutations cause Myelodysplastic Syndrome?

A

Driver mutations because it is a CLONAL disorder

27
Q

RAEB 1

A
  • seen with Myelodysplastic Syndrome

5%-9% increase in blasts

28
Q

RAEB 2

A
  • seen with Myelodysplastic Syndrome

10% - 19% increase in blasts

29
Q

What does RAEB stand for?

A

Refractory Anemia with Excess Blasts

- Used with Myelodysplastic Syndrome

30
Q

What drives proliferation with Myeloproliferative Neoplasms?

A

Constitutively active mutated Kinases

31
Q

Describe the overall pathway of what can occur with Myeloproliferative Neoplasms

A
  • Something in bone marrow proliferates
  • Neoplastic stem cells travel to other sites
  • Extramedullary Hematopoiesis occurs
    = Fibrosis and Acute Leukemias occur
32
Q

With Chronic Myeloid Leukemia it is a Myeloproliferative Neoplasm. What cell type proliferates?

A

Mature myeloid cells

33
Q

What translocation is present with Chronic Myeloid Leukemia?

A

t(9;22) - BCR-ABL

34
Q

t(9;22) causes a ______ to be constitutively active

A

Tyrosine kinase

35
Q

What 4 things can classify Chronic Myeloid Leukemia?

A

Marked Leukocytosis (WBC >100K)
Increased Buffy coat
Splenomegaly
Extramedullary Hematopoiesis

36
Q

What are the 3 phases of CML (Chronic Myeloid Leukemia)?

A

Chronic phase
Accelerated phase
Blast phase

37
Q

Blasts increase towards what phase of Chronic Myeloid Leukemia?

A

Towards the Blast (last) phase

38
Q

Myelofibrosis

A

Stroma proliferates

39
Q

Polycythemia Vera

A

RBC proliferates

40
Q

Essential Thrombocythemia

A

Platelets proliferate

41
Q

What mutations are drivers for growth for myelofibrosis, polycythemia vera, essential thrombocythemia?

A

JAK2 Kinase mutations

42
Q

Myelofibrosis affects what organ mostly?

A

Spleen

43
Q

Clonal proliferations (neoplasms) of immature dendritic cells

A

Langerhans cell Histiocytosis

44
Q

Langerhans cells are what type of cells?

A

Immature dendritic cells

45
Q

What 2 things can be seen with Langerhans cell Histiocytosis?

A
  1. Grooved nuclei of langerhans cells

2. Tennis racket shaped Birbeck granules

46
Q

Grooved nuclei of Langerhans cells and Birbeck granules classify?

A

Langerhans cell Histiocytosis

47
Q

There may also be lots of _____ with Langerhans cell Histiocytosis

A

Eosinophils

48
Q

What markers are used to identify Langerhans cell Histiocytosis?

A

S-100

CD1a

49
Q

What causes reactive splenitis (splenomegaly)?

A

Viral infection

50
Q

What causes congestive splenomegaly?

A

Hepatic dysfunction (bc of portal vein)

51
Q

What is Hypersplenism (splenomegaly) associated with?

A

Cytopenias

52
Q

What usually causes a splenic rupture?

A

Blunt trauma to abdomen

53
Q

Splenic neoplasms are often involved with ______ malignancies

A

Hematologic

54
Q

How easy is it for a splenic infarct to occur?

A

Very easy - blood only has 1 way into the spleen

55
Q

Hyperplasia of the thymus and a Thymoma can both produce?

A

Autoantibodies

56
Q

If Hyperplasia of the thymus or a Thymoma produce autoantibodies, what results?

A

Myasthenia gravis

57
Q

Symptoms with Myasthenia Gravis

A

Muscle weakness that gets worse with activity

SBL (48 decks)

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