Pathology Of The Bone Marrow Stem Cell Disorders

Front 1

What are the different clonal neoplastic disorders of the bone marrow?

Back 1

Myelodysplastic syndromes
Chronic myeloproliferative disorders
Acute leukemia

Front 2

What occurs in aplastic anemia?

Back 2

Lack of stem cells to keep the peripheral counts at the levels that they need to remain

Front 3

What is a common finding in both aplastic anemia and the myelodysplastic syndromes?

Back 3

Cytopenias

Front 4

What are the findings in aplastic anemia?

Back 4

Pancytopenia with bone marrow hypocellularity

Front 5

What are the serious complications of aplastic anemia?

Back 5

Infection (neutropenia)
Anemia
Bleeding (thrombocytopenia)

Front 6

What are the causes of aplastic anemia?

Back 6

50% idiopathic

50%: various different bone marrow toxins

Front 7

How do you classify the different types of myelodysplastic syndromes?

Back 7

Number of blasts
Severity of anemia
Severity of thrombocytopenia

Front 8

What finding is this? What is it a signal of?

Back 8

Ring sideroblast

It's a sign of myelodysplastic anemia

Front 9

What is the cell type on the left? What disorder is present on the right?

Back 9

Neutrophil on the left

On the right: myelodysplastic syndrome with dysplastic neutrophils

Front 10

What kind of cell is this here? What disorder is present at right?

Back 10

Megakaryocyte

On right: myelodysplastic megakaryocyte

Front 11

What's a difference between chronic myeloprolieferative and myeloblastic syndromes?

Back 11

CMPD: elevated peripheral counts

Myelodysplastic: decreased peripheral gcounts

Front 12

What are some other items on the differential for CMPDs?

Back 12

Neutrophilia/leukemoid reaction
Secondary polychthemia
Reactive thrombocytosis

Front 13

What is the leukemoid reaction?

Back 13

Reaction to a huge infection; can look like CMPD

Differences (in leukemoid reaction)
-You see toxic granulation and Dohle bodies in neutrophils
-Normal cytogenitics

Front 14

What is secondary polycythemia?

Back 14

People live at high elevations-->push reticulocytes out into the peripheral blood due to the chronic hypoxia

Front 15

What are Dohle bodies?

Back 15

RER inside the cytoplasm

Front 16

What types of cells are the chronic myeloproliferative disorders associated with? What is the course?

Back 16

Cells: more differentiated leukocytes

Course: indolent

Front 17

What types of cells are the acute myeloid and acute lymphoid leukemias associated with? What's the course?

Back 17

Cells: increased proportion of very immature cells (blasts)

Course: rapidly fatal!

Front 18

What are the chronic myeloproliferative disorders?

Back 18

Chronic myelogenous leukemia
Polycythemia vera
Primary myelofibrosis
Essential thrombocythemia

Front 19

What types of cells are found in chronic myelogenous leukemia?

Back 19

Blood, bone marrow proliferation of differentiated myeloid cells with left shift

Few blasts!

Basophilia in the blood and bone marrow; way more than you'd expect to see!

Front 20

What type of chromosome is found in chronic myelogenous leukemia?

Back 20

The Philadelphia chromosme

Balanced translocation between 22 and 9

Front 21

What is the dominant cell type in CML? What's the originating cell line?

Back 21

Dominant: granulocytic elements

Originating: multipotent stem cell

Front 22

What's the natural history of CML?

Back 22

1. Accelerated phase
2. Blast phase: increased proportion of the blasts--> gets worse faster.
-Some look like myeloid; some look like lymphoid

Front 23

What are the findings in polycythemia vera?

Back 23

Increase in hemoglobin and hematocrit
Hypercellular bone marrow
Moderate leukocytosis/thrombocytosis

Decreased erythropoietin levels: hormone loop is intact

Front 24

What mutation is present in PCV?

Back 24

JAK2 mutation

Front 25

What are complications of polycythemia vera?

Back 25

Thrombotic, hemorrhagic complications
Distension of blood vessels
Platelet dysfunction

Front 26

What's the late phase of polycythemia vera?

Back 26

Primary myelofibrosis

Front 27

What are the findings in primary myelofibrosis?

Back 27

Bone marrow becomes fibrotic, hypocellular
Progressive increase in extramedullary hematopoiesis (spleen, liver); splenomegaly
Variable counts

Front 28

What doe s a blood smear of primary myelofibrosis look like?

Back 28

Leukoerythroblastic peripherral blood smear

Front 29

What mutation is present in primary myelofibrosis?

Back 29

JAK2

Front 30

What type of cells go HAM in primary myelofibrosis?

Back 30

Fibroblasts!

You have fibrosis happen to the bone as an endpoin.

Front 31

What happens in primary myelofibrosis?

Back 31

You have marrow elements going to the other organs after they hit the bones.

Front 32

What is the characteristic finding in primary myeloid fibrosis?

Back 32

Teardrop erythrocytes!

Front 33

What is essential thrombocytopenia?

Back 33

Sustained platelet count > 450E9

Front 34

What are the cytogenetic findings in essential thrombocytopenia?

Back 34

No philadelphia chromosome
No marrow fibrosis

JAK2 mutation in 50% of cases

Front 35

What are the findings in a smear for essential thrombocytopenia?

Back 35

Abnormal megakaryocytes
Abnormal platelets!

Front 36

What are the findings in the acute leukemias?

Back 36

Bone marrow is largely populated by blasts
Leukemic blasts lead to an elevated WBC
Leukemic cells invade other tissues

Front 37

What's the definition of an acute leukemia?

Back 37

20-30% blasts in the acute leukemia

Front 38

What is the definition of acute lymphoblastic leukemia?

Back 38

>20% lymphoblasts in the bone marrow and/or peripheral blood

Front 39

What are the different subtypes of ALL?

Back 39

B cell
T cell

Front 40

What's a sign of a poor prognosis in ALL?

Back 40

Philadelphia chromosome t(9;22)

Front 41

What do you see in the marrow for people with ALL?

Back 41

Monotonous bone marrow biopsies and smears: all blasts!

Blasts:
-Larger
-Irregular
-Chromatin in the nuclei that you can see well

Front 42

What is the definition of acute myeloblastic leukemia?

Back 42

>20% myeloblasts in the marrow or peripheral blood

They've got cytoplasmic granules and Auer rods

Front 43

Who gets acute myeloblastic leukemia?

Back 43

Adults > kids

Front 44

What is an Auer rod? Where is it found?

Back 44

It's found in acute myeloblastic leukemia

Crystallized myeloperoxidase

Front 45

What the hallmark findings of FAB M3-acute myeloblstic leukemia?

Back 45

Distinctive cells with lots of Auer rods
Characteristic chromosomal translocation t(15;17)
High risk of DIC

Front 46

What's the treatment for acute myeloblastic leukemia?

Back 46

Retinoic acid!

Front 47

What stain do we use for granulocytic differentiation?

Back 47

Myeloperoxidase

Front 48

What stain do we use for monocytic differentiation?

Back 48

Nonspecific esterase

Front 49

How do we differentiate between AML and ALL?

Back 49

Flow cytometry