Red Blood Cells

Question 1

What are the lab findings in iron deficiency anemia?

Answer 1

1. microcytic hemochromic anemia
2. dec Ferritin, inc TIBC
3. dec serum iron
4. dec % saturation
5. inc RDW
6. inc FEP - protoporphyrin is free

Question 2

What is plummer vinson syndrome?

Answer 2

iron deficiency anemia with esophageal web and atrophic glossitis

Question 3

What are the lab findings of anemia of chronic disease

Answer 3

1. inc ferritin, dec TIBC
2. dec serum iron
3. dec % saturation
4. inc FEP
5. microcytic anemia

Question 4

What are the lab findings of sideroblastic anemia?

Answer 4

1. inc ferritin, dec TIBC
2. inc serum iron
3. inc % saturation
4. microcytic anemia

Question 5

In which microcytic anemia would exogenous EPO be useful?

Answer 5

anemia of chronic disease

Question 6

In which disease are heinz bodies formed? What are they made of?

Answer 6

Hemoglobin H disease

• 3 alpha genes are deleted
• tetramers of Beta chains

Question 7

In which diseases are HbA2 increased? What is HbA2 composed of?

Answer 7

beta thalassemias

-alpha 2, delta 2

Question 8

In which thalassemia is there extramedullary hematopoeisis?

Answer 8

B thalassemia major

- skull and facial bones: crew cut and thickened face

Question 9

where are the following nutrients absorbed:

1. iron
2. folate
3. vitamin b12

Answer 9

1. deuodenum
2. jejunum
3. ileum with intrinsic factor

Question 10

How is a deficiency of folate or vitamin B12 distinguished besides measuring the serum levels of both?

Answer 10

methylamnoic acid inc in vit B12 deficiency and NOT in folate

-build up of methylamnoic acid causes degeneration of the spinal cord

Question 11

Which type of hemolytic anemia results in jaundice?

Answer 11

extravascular

-build up of unconjugated bilirubin

Question 12

What three inherited defects are associated with hereditary spherocytosis?

Answer 12

membrane tethering proteins:

-ankyrin, spectrin, band 3

Question 13

What is used to diagnose inherited spherocytosis?

Answer 13

osmotic fragility test - inc fragility in hypotonic solution

Question 14

What type of cells are seen wtih acanthocytosis and what is usually the cause?

Answer 14

irregular spaced spiny projections

-chronic liver disease: inc cholesterol

Question 15

What is the amino acid replacement in sickle cell anemia?

Answer 15

glutamic acid is switched to valine which is hydrophobic

Question 16

What is the most common cause of death in children with sickle cell anemia?

Answer 16

infection with encapsulated organisms
-this is due to the autosplenectomy that results from sickle cell - shrunken fibrotic spleen - and therefore no antibody production occurs

Question 17

What specific infection are patients especially at risk for with sickle cell disease?

Answer 17

salmonella paratyphi osteomyelitis

Question 18

What is the most common cause of death in adults with sickle cell disease?

Answer 18

acute chest syndrome

Question 19

What type of anemia is present in patients with sickle cell disease?

Answer 19

mostly extravascular hemolytic anemia –> normocytic

-some intravascular is present

Question 20

In general when are howell jolly bodies seen on blood smear?

Answer 20

when the spleen is absent or defective

Question 21

What is the one location in the body patients with sickle cell trait are susceptible to sickling and ischemia as a result?

Answer 21

renal medulla: hypoxia, hypertonicity

• ischemic infarcts
• eventually leads to an inability to concentrate urine

Question 22

which amino acid is replaced in hemoglobin C?

Answer 22

glutamic acid –> lysine

Question 23

What is the defect in paroxysmal nocturnal hemoglobinuria?

Answer 23

ACQUIRED defect in GPI

-no protection from complement

Question 24

Why does intravascular hemolysis occur at night in patients with paroxysmal nocturnal hemoglobinuria?

Answer 24

acidosis at night - shallow breathing

-this activates complement

Question 25

What are some lab results of intravascular hemolysis?

Answer 25

• hemoglobinuria
• hemoglobinemia
• hemosiderinuria
• decreased haptoglobin

Question 26

what test is used to diagnose paroxysmal nocturnal hemoglobinuria?

Answer 26

sucrose test - activates complement

Question 27

What is the usual cause of death in paroxysmal nocturnal hemoglobinuria?

Answer 27

thrombosis of hepatic, portal, cerebral veins

-destroyed platelets release cytoplasmic contents leading to coagulation

Question 28

What are two lab findings that would be visualized by microscopy in G6PD deficiency?

Answer 28

• heinz bodies

- bite cells

Question 29

Describe the main differences between the two types of immune mediated hemolytic anemias

Answer 29

IgG mediated: extravascular

• warm central body
• associated with SLE, CLL, drugs
• can occur with maternal antibodies as they can cross the placenta

IgM mediated: intravascular

• cold peripheral body
• associated with mono and mycoplasma

Question 30

What does parvovirus infect?

Answer 30

progenitor red cells

-> anemia in the setting of preexisting marrow stress

Question 31

What is fanconi anemia?

Answer 31

• inherited autorecessive BM failure disorder - genes involved in DNA repair and genetic stability
• thrombocytopenia and RBC macrocytosis

Question 32

In which diseases are there target cells?

Answer 32

1. B thalessemia

2. sickle cell anemia

Question 33

Besides the hereditary causes of spherocytes, what is another cause of spherocytosis?

Answer 33

autoimmune hemolytic anemia

Question 34

In chronic renal disease, what are two pathological types of RBCs usually seen?

Answer 34

burr cells - scalloped

shistocytes - malignant htn

Question 35

What is the schilling test?

Answer 35

tests for if malabsorption is causing vit B12 deficiency

Question 36

In which hereditary cause of normocytic anemia, is a complication of gallstones very prominent?

Answer 36

hereditary spherocytosis

Question 37

Patients with paroxysmal nocturnal hemoglobinuria are at an increased risk for what disease?

Answer 37

AML