Heme/Onc

Question 1

Which blood cells are produced in the myeloid lineage?

Answer 1

RBCs
granulocytes: neutrophils, basophils, eosinophils
Platelets
Monocytes

Question 2

What blood cells are produced in the lymphoid lineage?

Answer 2

B & T lymphocytes

Question 3

What are causes of neutropenia?

Answer 3

Drug toxicity (chemo)
Severe infection

Question 4

How can you treat neutropenia in a chemo patient?

Answer 4

GM-CSF

G-CSF

Question 5

What are causes of lymphopenia?

Answer 5

Immunodeficiency (DiGeorge syndrome)
High cortisol state (Cushings)
Autoimmune destruction (SLE)
Whole body radiation (lymphocytes most sensitive to radiation)

Question 6

What are causes of neutrophilic leukocytosis?

Answer 6

Bacterial infection
Tissue necrosis
High cortisol state

Question 7

What is the importance of left shift neutrophilia?

What cell markers are decreased compared with mature neutrophils?

Answer 7

Many immature neutrophils leaving the circulation

Immature neutrophils have decreased Fc receptors (don’t work as well) as seen by decreased expression of CD16

Question 8

What causes a monocytosis?

Answer 8

chronic inflammation

malignancy

Question 9

What causes eosinophilia?

Answer 9

Allergic reactions
Parasitic infection
Hodgkin lymphoma (increased IL-5 production)

Question 10

What causes basophilia?

Answer 10

CML

Question 11

What causes lymphocytosis?

Answer 11

Viral infections

Bordetella pertussis

Question 12

What are some clinical features of a CD8+ T cell response? (EBV infection)

Answer 12

generalized lymphadenopathy (paracortex)
splenomegaly (periarterial lymphatic sheath-PALS)
high WBC with atypical lymphocytes

Question 13

How does a monospot test work?

Answer 13

Detects IgM heterophile antibodies
(takes 1 week after infection to test positive)
**screens but doesn’t diagnose Mono

Question 14

How do you test to diagnose EBV

Answer 14

EBV viral capsid antigen

screening test is monospot/heterophile antibody test

Question 15

What is a long term complication of EBV infection?

Answer 15

Higher risk of lymphoma if dormancy of virus in B cells

Question 16

What cells types are most numerous in acute leukemia?

Answer 16

Immature (blast) cells

>20% blasts, that crowd out normal cells that result in anemia, thrombocytopenia or neutropenia

Question 17

What do myeloid/lymphoid blasts cells look like (blood smear)?

Answer 17

Large, punched out nucleus (nucleolus), immature (very little cytoplasm)

Question 18

What are the cell markers on myeloblasts?

Answer 18

myeloperoxidase (MPO)

crystalizes into Auer rods

Question 19

What are the cell markers on lymphoblasts?

Answer 19

TdT+ on nuclear staining (DNA polymerase)
B-ALL: CD10, CD19, CD20
T-ALL: CD2-8 NOT CD10

Question 20

What are some genetic translocations associated with B-ALL (prognosis?)

Answer 20

t(12;21) – good prognosis, commonly seen in kids

t(9;22) – poor prognosis, commonly seen in adults

Question 21

What disease has t(12;21)?

Answer 21

B-ALL, usually kids with good prognosis

Question 22

What disease has t(9;22)?

Answer 22

B-ALL, usually adults with poor prognosis Ph+ALL

CML

Question 23

What are the features of T-ALL?

Answer 23

Thymic mass (mediastinal lymphoma)
Teenagers
TdT+, and CD2-8, NOT CD10

Question 24

How can you tell blasts cells are AML?

Answer 24

MPO on stain or crystalization into Auer rods

Question 25

What disease has t(15;17)?

Answer 25

AML

Question 26

What is the result to this translocation? | t(15;17)

Answer 26

Disrupts retinoic acid receptor --> promyelocytes (containing Auer rods) accumulate --> risk for DIC Tx: ATRA causes cells to mature

Question 27

What is basophilic stippling and when do you see it?

Answer 27

Basophilic granules in RBCs - Anemia of Chronic disease - alcohol abuse - lead poisoning - thalassemias

Question 28

What are acanthocytes and when do you see them?

Answer 28

Spiny RBCs (spur cells) - liver disease - abetalipoproteinemia (cholesterol dysregulation)

Question 29

What are bite cells and when do you see them?

Answer 29

RBCs that look like they have a bite taken out | - G6PD deficiency

Question 30

What do you think when there are big, oval shaped RBCs on blood smear?

Answer 30

Macro ovalocytes - megaloblastic anemia (w/ hypersegmented PMNs) - marrow failure

Question 31

What are ringed sideroblasts and when do you see them?

Answer 31

Sideroblastic anemia | Excess iron in mitochondria

Question 32

What are schistocytes and when do you see them?

Answer 32

Darth Vader helmet cells - DIC - TTP/HUS - traumatic hemolysis (mechanical heart valves)

Question 33

What do you think when you see spherocytes on blood smear?

Answer 33

Hereditary spherocytosis | Autoimmune hemolysis

Question 34

What do you think when you see teardrop cells on blood smear?

Answer 34

Bone marrow infiltration (myelofibrosis)

Question 35

What do you think when you see target cells on blood smear?

Answer 35

HbC disease Asplenia Liver disease Thalassemia "HALT!" Said the hunter to his TARGET

Question 36

What are Howell-Jolly bodies and when would you see them?

Answer 36

Basophilic nuclear remnant in an RBC - functional hyposplenia - asplenia

Question 37

What are Heinz bodies and when would you see them?

Answer 37

Hgb precipitation from oxidation of sulfhydryl groups in an RBC (seen in crystal violet stain) - G6PD deficiency (w/ quinolones) - alpha-thalassemia * **phagocyte damage may turn them into bite cells

Question 38

What are the signs of multiple myeloma?

Answer 38

CRAB: Calcium (high) from increased bone breakdown Renal failure Anemia Bone lesions (lytic in skull and axial skeleton) Protein (high in serum) from excess Igs

Question 39

What are Bence Jones proteins?

Answer 39

Free light chains: immunoglobulin precipitates into tubular cast in the renal tubules caused by myeloma nephrosis

Question 40

What is Hemophilia A?

Answer 40

``` Deficiency of factor VIII prolongs PTT (intrinsic pathway) ```

Question 41

What is Hemophilia B?

Answer 41

``` Deficiency of factor IX prolongs PTT (intrinsic pathway) ```

Question 42

What is Bernard-Soulier syndrome?

Answer 42

Defect in platelet plug formation | decreased GpIb --> defect in platelet to vWF adhesion

Question 43

What is Glanzmann thrombasthenia?

Answer 43

Defect in platelt plug formation decreased GpIIb/IIIa --> defect in platelet to platelet aggregation **no platelet clumbing on blood smear

Question 44

What is Immune thrombocytopenia?

Answer 44

anti-GpIIb/IIIa antibodies --> splenic macrophage consume platelet antibody complex **decreased platelet survival (low number) megakaryocytes on bone marrow biopsy

Question 45

What is thrombotic thrombocytopenic purpura?

Answer 45

deficiency or inhibition of vWF metalloprotease (ADAMTS 13) --> decreased degradation of vWF multimers --> platelet adhesion/aggregation --> thrombosis **schistocytes, increased LDH

Question 46

What are some symptoms of TTP?

Answer 46

fever thrombocytopenia microangiopathic hemolytic anemia neruologiv and renal symptoms