11/2 - Hematopoietic and Lymphoid System

Question 1

what is reduction of the total circulating red cell mass below normal limits

Answer 1

anemias

Question 2

what reduce the oxygen carrying capacity of blood, leading to tissue hypoxia

Answer 2

anemias

Question 3

what is anemia diagnosed by

Answer 3

1. hematocrit
2. hemoglobin concentration of the blood

Question 4

what is the life span of RBC

Answer 4

Question 5

what is the ratio of packed RBC to total blood volume

Answer 5

hematocrit

Question 6

what are small red cells with decreased mean corpuscular volume (MCV)

Answer 6

microcytic

Question 7

what are large red cells with increased MCV

Answer 7

macrocytic

Question 8

what are pale red cels with decreased mean corpuscular hemoglobin (MCH)

Answer 8

hypochromic

Question 9

what are oddly shaped cells

Answer 9

poikilocytosis

Question 10

what is mixture of red cell sizes

Answer 10

anisocytosis

Question 11

what are bulls-eye red cells

Answer 11

target cells

Question 12

symptoms of anemia

Answer 12

1. Fatigue, lack of exercise tolerance
2. May have cardiac pain (angina) due to increased heart rate and decreased oxygenation (coronary artery disease)
3. Pale skin and pale mucous membranes
4. Atrophic glossitis; usually in severe cases only

Question 13

classifications of anemia due to underlying mechanism

Answer 13

I. Blood loss
2. Increased red blood cell destruction (hemolysis)
3. Decreased red cell production

Question 14

what are examples of acute and chronic blood loss that causes anemia

Answer 14

1. acute blood loss = trauma
2. chronic blood = GI tract lesions, gynecological disturbamces

Question 15

mechanism and examples of increased red cell destruction and specific examples

Answer 15

Question 16

mechanism and examples of decreased red cell production

Answer 16

Question 17

hemoglobin abnormalities that cause increased red cell destruction

Answer 17

1. sickle cell anemia
2. thalassemia

Question 18

what is a group of hereditary disorders characterized by the presence of a structurally abnormal hemoglobin

Answer 18

hemolytic anemia of hemoglobin abnormalities

Question 19

what result in decreased red cell survival and often acute hemolysis under certain biochemical and physiologic stress

Answer 19

hemolytic anemia of hemoglobin abnormalities

Question 20

what is single amino acid substitution, valine for glutamic acid, in the beta globin chain; worldwide, most common form of familial hemolytic anemia

Answer 20

sickle cell anemia

Question 21

8% of who is heterozygous for HbS

Answer 21

african americans

Question 22

is sickle cell traitAR or AD

Answer 22

AR

Question 23

when does HbS polymerize

Answer 23

when deoxygenated and crystallizes producing the characteristic sickle shape

Question 24

what amino acid is replaced in sickle cell anemia

Answer 24

glutamic acid becomes VALINE

Question 25

what chain is the problematic chain in sickle cell anemia

Answer 25

beta chain

Question 26

is sickle cell trait heterozygous or homozygous

Answer 26

heterozygous

Question 27

is sickle cell anemia heterozygous or homozygous

Answer 27

homozygous sickle cell disease

Question 28

what this: One inherited copy of a genetic variant of hemoglobin S (HbS) 41% or less of Hemoglobin S Usually, no symptoms; immense stress on body precipitates sickling of RBCs

Answer 28

sickle cell trait

Question 29

what this: Two inherited copies of HbS Most common form of sickle cell disease Requires ongoing medical treatment; acute pain is so severe that it is called "crisis"

Answer 29

sickle cell anemia

Question 30

effects of sickling

Answer 30

1. reducing life space of RBC from 120 to 20 2. autosplenectomy 3. may cause widespread mirovascular obstructions 4. bone infarcts common 5. impaired immune response

Question 31

what happens when disease (e.g., sickle cell anemia) damages the spleen to such an extent that it becomes shrunken and nonfunctional

Answer 31

autosplenectomy

Question 32

oral manifestatoins of sickle cell anemia

Answer 32

1. orofacial and dental pain 2. pallor of oral mucosa and delayed tooth eruption 3. **radiographic: stepladder trabecular pattern**

Question 33

what disease has step ladder pattern in premolar and molar areas as an oral manifestation

Answer 33

sickle cell anemia

Question 34

how to treat oral manifestation of sickle cell anemia

Answer 34

long term penicillin prophylaxis significantly lowers caries in children with SCA

Question 35

treatment for sickle cell trait

Answer 35

no treatment required

Question 36

treatment for sickle cell anemia

Answer 36

Pain medications Oxygen therapy Hydroxyurea and crizanlizumab (prevents vaso- occlusive crisis) Bone marrow transplant Oxbryta/ Voxelotor (prevents Hb from polymerizing) Gene therapy (LentiGlobin)- clinical trials

Question 37

what gene mutation for sickle cell disease

Answer 37

enzyme pyruvate kinase (missing) for gene PLKR