Hematopathology

Question 1

What is hematopoiesis?

Answer 1

Production of blood cells and formed elements in blood

Question 2

Where does hematopoiesis take place during the fetal period?

Answer 2

Liver and spleen

Question 3

Where does hematopoiesis take place during the neonatal period and into adult life?

Answer 3

Bone marrow compartment

Question 4

What is a committed stem cell?

Answer 4

Myeloid or erythroid

Question 5

Where do committed stem cells come from?

Answer 5

Multipotent stem cells

Question 6

What are some different methods to evaluate blood cells and the marrow compartment?

Answer 6

1) Peripheral blood collection and smear
2) Bone marrow biopsy, aspirate and smear
3) Flow cytometry

Question 7

What is anemia?

Answer 7

A reduction below normal in the volume of packed red cells, as measured by the hematocrit, or the reduction in the hemoglobin concentration of the blood

Question 8

What are some causes of macrocytic anemia? And what is it?

Answer 8

Macrocytic anemia is an enlargement of RBCs. This is usually due to the lack of B12 or folate. These cells get bigger and bigger to divide but because they are lacking these essential vitamins they cannot divide.

Question 9

What are some causes of microcytic hypochromic anemia and what is it?

Answer 9

These cells are smaller and lack a certain amount of hemoglobin leading to the paler color in the RBC. These cells are usually caused by Fe (iron) deficiency, thalassemia, and ACD.

Question 10

What is a normal range for iron content for women and men?

Answer 10

Women: 2-4 g
Men: 6g

Question 11

What percentage of iron is found in hemoglobin, myoglobin and iron containing enzymes in the body?

Answer 11

80%

Question 12

What percentage of iron is in storage pools?

Answer 12

20%

Question 13

What are storage pools for iron?

Answer 13

Ferritin and hemosiderin

Question 14

________ is a good indicator of the adequacy of body iron stores.

Answer 14

Ferritin

Question 15

How is iron transported in the plasma?

Answer 15

By a protein known as transferrin. Absorbed iron is taken by portal blood to liver and bone marrow

Question 16

Where is heme usually absorbed?

Answer 16

Duodenum

Question 17

What are some indicators to diagnose iron deficiency anemia?

Answer 17

1) Hypochromia, polychromasia, and microcytosis on a peripheral smear
2) Decreased MCV
3) Decreased serum iron
4) Decreased ferritin (cell and plasma)
5) Increased total iron binding capacity (TIBC)

Question 18

What are some clinical features associated with iron deficiency anemia?

Answer 18

1) Koilonychias - abnormally thin, flat nails
2) Cheliosis - inflammation of one or more corners of mouth
3) Glossitis - inflammation with depapillation of the dorsal surface of the tongue; sometimes soreness

Question 19

What is pernicious anemia?

Answer 19

Anemia due to B12 deficiency - causes megaloblastic cells

Question 20

What is another word for B12?

Answer 20

Methylcobalamin

Question 21

What does B12 do?

Answer 21

Converts homocysteine to methionine and the reaction yields FH4 (tetrahydrofolic acid)

Question 22

How is B12 liberated?

Answer 22

From protein by pepsin

Question 23

What does B12 bind to in saliva?

Answer 23

R-binder

Question 24

R-B12 is broken down by pancreatic enzymes and is released then binds to ___________________which is produced by parietal cells.

Answer 24

Intrinsic factor

Question 25

IF-B12 complex travels to the __________ where receptors for IF are located.

Answer 25

ileum

Question 26

The IF-B12 complex is absorbed by mucosa and travels through blood by ______________________.

Answer 26

transcobalamin II

Question 27

What is the cause for Chronic Atrophic Gastritis?

Answer 27

The gastric mucosa is destroyed by a parietal cell antibody that blocks the B12-IF binding, so B12 can't be carried to the ileum. This causes inflammation and loss of parietal cells

Question 28

What are some diagnostic features involving megalobastic anemia?

Answer 28

1) Leukopenia with hypersegmented granulocytes 2) Thrombocytopenia 3) Neurological changes associated with posterolateral columns 4) Inability to absorb and oral dose of cabalamin 5) Low serum B12

Question 29

If a patient has low serum B12 and is unable to absorb an oral dose of cobalamin, what might the patient have?

Answer 29

Megaloblastic anemia

Question 30

Folic acid deficiency is sometimes seen in ______________.

Answer 30

Alcoholics

Question 31

T/F - Humans are entirely dependent on dietary sources for folic acid.

Answer 31

True

Question 32

How can you tell the difference between pernicious anemia and folic acid deficiency?

Answer 32

Pernicious anemia shows neurological symptoms, poor balance, and problems with steriognosis. Folic acid deficiency has NO neurological symptoms.

Question 33

In order to diagnose Pernicious anemia, a ______________ test should be done. Folic acid levels should be tested for folic acid deficiency.

Answer 33

Schilling Test

Question 34

What is the major repository for mononuclear phagocytic cells?

Answer 34

Spleen - It is the recycling center for RBCs (iron), filtration organ of unwanted elements from the blood, a major secondary organ, reserve pool and storage site, and also a source of lymphoreticular cells.

Question 35

What disease is seen to have premature destruction of RBCs, accumulation of products of hemoglobin catabolism, and compensatory increase in erythropoiesis within bone marrow?

Answer 35

Hemolytic anemia

Question 36

_________________ hemolysis causes damage to RBCs be mechanical, immune, or toxic factors.

Answer 36

Intravascular

Question 37

_________________ hemolysis occurs whenever red cells are injured, rendered foreign or become less deformable and are sequestered by the spleen for destruction.

Answer 37

Extravascular

Question 38

What occurs in G6P dehydrogenase (G6PD) deficiency?

Answer 38

G6PD regenerates NADPH, which generates GSH, and H2O2 disposal is dependent on GSH. So, if G6PD is deficient, there is lack of GSH to dispose of the H202. This causes cell injury due to the oxidant free radicals (H2O2). Heinz bodies are seen due to the denatured globin within the cell.

Question 39

What are some key features seen in sickle cell anemia?

Answer 39

- Hereditary - Abnormal hemoglobin - Beta-globulin gene - Substitution of Valine for Glutamic acid at 6th position Beta-globulin chain

Question 40

Sickle cell hemoglobin is also known as _____.

Answer 40

HbS

Question 41

What occurs to the RBCs of a patient with sickle cell anemia?

Answer 41

HbS molecules aggregate and polymerize upon deoxygenation leading to the formation of HbS fibers and distortion of the RBCs. Repeated episodes of sickling and unsickling, cause permanent damage, leading to irreversible sickled RBCs.

Question 42

Heterozygotes (sickle cell trait) RBCs sickle under conditions of severe __________.

Answer 42

Hypoxia

Question 43

Why don't newborns manifest the sickle cell disease until they are about 5-6 months?

Answer 43

Fetal hemoglobin inhibits the polymerization of HbS The reason that infants don’t show symptoms at birth is because baby or fetal hemoglobin protects the red blood cells from sickling. When the infant is around 4 to 5 months of age, the baby or fetal hemoglobin is replaced by sickle hemoglobin and the cells begin to sickle.

Question 44

What different things can be seen in sickle cell disease?

Answer 44

- Splenomegaly (reticulocyte entrapment within the spleen due to deformed RBCs) - Autosplenectomy (damage to spleen is so vast that the organ becomes so small and non-functional) - Secondary Hemochromatosis - Gallstones - Legulcers

Question 45

What are the ways to test for Sickle cell anemia?

Answer 45

- Peripheral blood smear - Hgb electrophoresis - Sickling test

Question 46

What is thalassemia?

Answer 46

Characterized by lack of or decreased synthesis of either alpha- or beta-globin chain of HbA