Leukemias

Question 1

Myeloid neoplasm categories:

Answer 1

Myeloproliferative Disorders
Myelodysplastic Syndromes
Acute Myeloid Leukemia

Question 2

Myeloproliferative categories:

Answer 2

CML
PV
PMF
ET

Question 3

What occurs in myeloproliferative neoplasms?

Answer 3

Increased production of one or more myeloid lineages

Question 4

Peripheral smear of myeloproliferative neoplasms:

Answer 4

Pancytosis

Question 5

Bone marrow of myeloproliferative neoplasms:

Answer 5

Hypercellularity leading to hypocellularity and fibrosis

Question 6

What occurs in myelodysplastic syndromes?

Answer 6

Defective maturation of myeloid progenitors leading to cytopenias

Question 7

Myelodysplastic syndromes peripheral smear:

Answer 7

Dysplastic immature cytopenias less than 20%

Question 8

Myelodysplastic syndrome bone marrow biopsy:

Answer 8

Hypercellular with blasts less than 20%

Question 9

What occurs in AML?

Answer 9

Accumulation of immature myeloid forms (blasts) in bone marrow and blood leading to marrow suppression

Question 10

What causes CML?

Answer 10

9->22 Bcr-Abl constitutively active tyrosine kinase

Question 11

Hallmark seen in CML blood smear:

Answer 11

Basophilia and granulocyte increase

Question 12

Three phases of CML:

Answer 12

Chronic: blasts less than 10%
Accelerated: 10-19% lots of basophils
Blast phase: greater than 20% blasts

Question 13

Key features of myelodysplastic syndromes:

Answer 13

Cytopenias
Less than 20% blasts
Risk turn to AML
Pseudo-Pelger-Huet cells

Question 14

What mutation causes AML?

Answer 14

15 -> 17 forming PML/Rara protein; keeps cells from maturing

Question 15

What cells are seen in AML smear?

Answer 15

Auer Rods in promyelocytes

Question 16

Polycythemia vera cause:

Answer 16

JAK2 mutation so always on

Question 17

What is seen in PV?

Answer 17

Hgb greater than 18.5 or 16.5 with O2 saturation greater than 92 and low EPO levels

Question 18

Primary and secondary erythrocytosis:

Answer 18

Primary: autonomous proliferation of progenitor cells without EPO
Secondary: increased plasma EPO levels

Question 19

PV presentation:

Answer 19

Aquagenic pruritus
Erythromelalgia
Hyperuricemia
H. pylori infections

Question 20

What can PV turn to?

Answer 20

AML or myelofibrosis

Question 21

What gene mutations can cause essential thrombocythemia?

Answer 21

JAK2
CALR
MPL

Question 22

What happens in ET?

Answer 22

Clonal HSC disorder causing thrombocytosis

Question 23

Clinical consequences of ET?

Answer 23

Arterial thrombosis
Venous thrombosis
Erythromelalgia
Hemorrhage (if platelets are made wrong)

Question 24

What will be seen in BM biopsy of ET?

Answer 24

Megakaryocyte clustering and nuclear hyperlobulation

Question 25

What will be seen in ET peripheral blood?

Answer 25

Hyperlobulated large megakaryocytes

Question 26

Diagnostic criteria for ET?

Answer 26

1.) Sustained elevated platelet count; Pathogenic mutation; No other myeloid malignancy
or
2.) Sustained elevated platelet; No other myeloid malignancy; No cause for thrombocytosis and normal iron stores; BM show increased large hyperlobulated megakaryocytes

Question 27

ET treatment:

Answer 27

Hydroxyurea: stops cells at G1/S phase

Question 28

What mutation causes primary myelofibrosis?

Answer 28

JAK2 or MPL or CALR

Question 29

Pathology of PMF?

Answer 29

Hematopoeitic cells enter megakaryocyte cytoplasm releasing granules to damage the megakaryocyte which then releases growth factors causing fibroblast activation and infiltration

Question 30

What ultimately happens in PMF?

Answer 30

Fibroblasts cause fibrosis of bone marrow by depositing collagen

Question 31

In PMF what does the fibrosis cause?

Answer 31

Crowding of BM and EMH occuring

Question 32

What is seen in PMF?

Answer 32

Megakaryocyte clumping

SPLENOMEGALY

Question 33

What is given to combat PMF? What do they do?

Answer 33

Thalidomide and Lenalidomide

Antiangiogenic properties to stop vascularity going to the fibrosis

Question 34

CBC in PMF:

Answer 34

Anemia
Leucocytosis
Tear-drop red cells

Question 35

BM in PMF:

Answer 35

Fibrotic marrow

Hypocellular

Question 36

MDS treatment:

Answer 36

Thalidomide analog lenalidomide if anemic and 5q- patient