Leukemias & Myeloproliferative Diseases

Question 1

Pancytopenia

Answer 1

Low RBCs, WBCs, Platelets

Question 2

Most childhood leukemia is what type?

Answer 2

Acute Lymphocytic Leukemia (ALL) - 80% of childhood leukemia is ALL but only 20% of adult leukemia is ALL

Question 3

Where is the malignancy in the cell lineage in the case of ALL?

Answer 3

ALL is a malignancy of progenitor cells in the lymphoid lineage (the lineage leading to B and T Cells)

Question 4

What causes ALL, AML?

Answer 4

Often exposure to chemo, radiation, benzene toxins, also unknown etiology. smoking in AML.

Question 5

ALL Labs will show:

Answer 5

Pancytopenia w/ circulating blasts & increased blast B and T cells in the hyper cellular bone marrow; thymus will be enlarged.

Question 6

Signs and symptoms of ALL, AML:

Answer 6

Rapid onset, bleeding bums/mucosa, epistaxis, infections, pallor, purpura & petechiae, ecchymosis, bone pain / tenderness

Question 7

ALL Treatment

Answer 7

Chemo / steroids, bone marrow transplant

Question 8

What is the cell lineage affected in acute myelogenous leukemia?

Answer 8

AML affects myeloid cell progenitors (granulocytes, WBCs, megakaryocytes, RBCs)

Question 9

What segment of the population usually gets AML?

Answer 9

Adults over 60.

Question 10

With labs and radiology, how do you differentiate AML from ALL?

Answer 10

ALL patients - enlarged mediastinal mass on x-ray; AML patients will have blast cells that present with AUER RODs (needle-like inclusions in the cytoplasm), cytoplasm w/granules.

Question 11

What is the most common type of leukemia / who gets it?

Answer 11

Chronic Lymphocytic Leukemia / adults & elderly

Question 12

What cells are affected in Chronic Lymphocytic Leukemia?

Answer 12

B Cells.

Question 13

Labs for CLL:

Answer 13

Normal hematocrit and platelet count

Question 14

Treatment for CLL:

Answer 14

In early stages - observe; in late stages - combination chemotherapy

Question 15

Who gets Chronic Myeloid Leukemia?

Answer 15

Middle Aged (avg. age of onset is 55)

Question 16

What occurs in CML?

Answer 16

Over production of myeloid progenitor cells

Question 17

CML and CLL can both remain ___________________ for years:

Answer 17

asymptomatic and low grade

Question 18

Etiology of CML:

Answer 18

Philadelphia Chromosome (translocation)

Question 19

Symptoms of CML:

Answer 19

Night sweats, priapism, fever, blurred vision, splenomegaly, sternal tenderness from bone marrow expansion

Question 20

Labs in CML:

Answer 20

Left shifted myeloid cells, elevated WBCs

Question 21

CML can progress to:

Answer 21

AML

Question 22

Polycythemia Vera is caused by:

Answer 22

Caused by mutation in signaling protein (JAK2)

Question 23

What occurs in polycythemia vera?

Answer 23

The body overproduces all types of blood cells, particularly RBCs. This leads to thicker blood viscosity.

Question 24

Symptoms in Polycythemia Vera:

Answer 24

Tinnitus, headache, dizziness, blurred vision, epistaxis, fatigue, pruritis due to increased histamine release, clots, splenomegaly.

Question 25

EPO level in a patient with polycythemia vera.

Answer 25

Even though all cells are increased, EPO is NOT increased.

Question 26

Polycythemia Vera labs:

Answer 26

EPO - normal, Increased RBC mass, elevated WBCs and platelets, splenomegaly, elevated hematocrit, high B-12 levels.

Question 27

Polycythemia Vera Treatment:

Answer 27

Phlebotomy

Question 28

Essential Thrombocytosis cause:

Answer 28

Unknown

Question 29

Essential Thrombocytosis labs:

Answer 29

High platelet counts, high megakaryocytes, normal RBC count, most everything else = normal

Question 30

Complications of Essential Thrombocytosis:

Answer 30

Clotting

Question 31

Most common symptoms (2) of Essential Thrombocytosis:

Answer 31

Burning of hands, splenomegaly

Question 32

Myeloproliferative Disorders:

Answer 32

Polycythemia Vera & Essential Thrombocytosis

Question 33

What disease can follow either of the two Myeloproliferative Diseases?

Answer 33

Myelofibrosis

Question 34

Myelofibrosis occurs secondary to:

Answer 34

It occurs secondarily to the two myeloproliferative disorders

Question 35

What causes myelofibrosis?

Answer 35

Increased secretion of platelet derived growth factors and cytokines

Question 36

Side effects of myelofibrosis:

Answer 36

splenomegaly and hepatomegaly due to extramedullary hematopoiesis in the liver, spleen, and lymph nodes.

Question 37

Symptoms of Myelofibrosis:

Answer 37

Insidious onset - abdominal fullness, anemia & fatigue, enlarged spleen and liver, fibrosis of bone marrow, thrombocytopenia leads to bleeding, cachectic, bone pain

Question 38

Lab work of Myelofibrosis always shows:

Answer 38

Anemic Triad

Question 39

What is the anemic triad?

Answer 39

Tear drop poikilocytosis, leukoerythroblastic blood, giant abnormal platelets

Question 40

Treatment for Myelofibrosis:

Answer 40

Stem cell transplant (only curative tx), splenectomy (not curative)

Question 41

Elevated WBCs with the presence of blasts is:

Answer 41

Acute leukemia - medical emergency - call 9-11.