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1
Q

Ferroxidase present on the basolateral membrane

A

Hephaestin

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2
Q

TrANSFERRIN SIZE AND WHERE IT IS PRODUCED

A

84 kd
lIVER
*** BINDING SPECIFICITY AND AFFINITY very high even at decreased pH

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3
Q

Hepcidin size and where it is produced

A

25 aa

liver

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4
Q

recycler of iron in the body

A

macrophage of the reticuloendothelium system

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5
Q

Following administration of supplemental iron, ___ will respond within hours, ___ will increase in 3-5 days, and ___ increases at about 10 days

A

serum iron
reticulocyte count
hemoglobin

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6
Q

Why do you continue iron treatment after hemoglobin is normal?

A

Need to increase ferritin stores

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7
Q

Mutation that is the major cause of iron overload

A

HLA-H gene

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8
Q

HLA-H gene

A

The HLA-H gene encodes
for a protein in the duodenal crypt cells which acts as a co-factor for an absorption resulting in an
increase in absorption of iron presented to the gut and accumulation of iron in tissues

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9
Q

Each RBC contains how many hemoglobin?

A

approximately 280 million

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10
Q

Size of hemoglobin

A

68 KD

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11
Q

Mnemonic to remember basic shape of hemoglobin dissociation curve

A

10-10 30-30 60-90 40-75

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12
Q

2,3 BPG

A

byproduct of anaerobic glycolytic pathway

stabilizes T conformation

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13
Q

Normal concentration of 2,3 BPG

A

5 mmol/L

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14
Q

Hemoglobin Gower I

A

Zeta2 Epsion2

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15
Q

Hgb Gower II

A

alpha2 epsilon2

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16
Q

Hgb Portland

A

Zeta2 gamma 2

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17
Q

Hemolytic anemia forms

A

Heinz bodies

denatured hemoglobin

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18
Q

Hgb Zurich

A

increases binding to CO

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19
Q

Maintains iton in ferrous form within erythrocytes

A

NADPH methemoglobin reductase

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20
Q

2 exposures that lead to methemoglobin

A
  1. Benzocaine (topical anesthetic)

2. well water contaminated with nitrates

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21
Q

Normal CO levels

A

3%

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22
Q

Smoker Co levels

A

10-15%

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23
Q

Erythrocyte LS in circulation

A

120 days

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24
Q

platelet LS in circulation

A

7-10 days

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25
Q

neutrophils ls in circulation

A

7 hour half life

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26
Q

Wright-stained reticulocyte

A

polychromatophilic

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27
Q

Pernicious anemia

A

autoimmune destruction of IF producing gastric parietal cells

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28
Q

Myloid to erythroid ratio megaloblastic anemias

A

erythroid predominance

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29
Q

Reaction involving B12 but not folate

A

synthesis of succinyl CoA from methymalonyl CoA

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30
Q

Schilling test

A
  1. give oral radiolabeled comalbumin
  2. comalbumin absorbed in terminal ilium and binds TcII
  3. Flushing dose given
  4. If less radiolabeled Cbl in urine, then positive test
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31
Q

Target cells are characteristic of

A

thalassemias and HbE diseases

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32
Q

Inheritance of sickle cell

A

autosomal recessive

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33
Q

Things that cause oxidative stress to RBCs (4)

A
  • sulfa drugs
  • antimalarials
  • infections
  • fava beans
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34
Q

4 clinical manifestations of sickle cell anemia

A
  1. increased retic count
  2. increased WBC and platelet count
  3. Increased RDW
  4. increased unconjugated bilirubin, LDH, and AST
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35
Q

Most common chronic cause of death in adults with sickle cell disease

A

PAH

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36
Q

Most acute cause of death in sickle cell diseasse

A

Acute chest syndrome

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37
Q

bilirubin is ___ soluble

A

lipid

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38
Q

More water soluble form of bilirubin

A

urobiligen

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39
Q

Fate of urobiliogen

A

enterohepatic circulation or excreted by the kidney into the urine

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40
Q

Direct antiglobin test (DAT) evaluates the presence of (3)

A

IgG, C3d, or C4d

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41
Q

Inheritance of hereditary spherocytosis

A

most are autosomal dominant

25% are autosomal recessive

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42
Q

3 vaccinations pre-splenectomy

A
  • H. influenza b
  • S. pnemoniae
  • meningococcus
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43
Q

After splenectomy

A
  • prophylactic antibiotics (penicillin)
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44
Q

G-6-PD provides resistence to

A

plasmodium vivax

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45
Q

With oxidant stress, denatured hemoglobin does what?

A

attaches to the membrane and damages spectrin

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46
Q

Pyruvate kinase converts ____ to ____

A

phosphoenolpyruvate to pyruvate

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47
Q

In PK deficiency, the decrease in conversion of phosphoenolpyruvate to pyruvate causes (5)

A

decreased ATP and increased 2,3- BPG, loss of membrane plasticity, increase in rigidity, destruction in spleen

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48
Q

Things that cause oxidative stress to RBCs (4)

A
  • sulfa drugs
  • antimalarials
  • infections
  • fava beans
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49
Q

Treg

A

make lymphokines that suppress activation of sibling T cells

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50
Q

Cold antibody autoimmune hemolytic anemia ___vascular hemolysis

A

intravascular

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51
Q

Warm antibodies

A

IgG

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52
Q

Warm antibodies ____vascular hemolysis

A

extravascular

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53
Q

Steps of cold antibody autoimmune hemolytic anemia

A
  • Cold antibodies bind RBCs in cooler areas of body
  • when cells move to central circulation, activate C5-C8 attack complex, which creates holes in the membrane
  • antibody dissociates because of low affinity at high temps and complement destroys cells
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54
Q

Chronic frustrated immune response

A

antigen not self, but something you can’t get rid of

EX: celiac and IBD

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55
Q

Total WBC

A

4.5-10.5 x 10^9/ L

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56
Q

3 vaccinations pre-splenectomy

A
  • H. influenza b
  • S. pnemoniae
  • meningococcus
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57
Q

After splenectomy

A
  • prophylactic antibiotics (penicillin)
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58
Q

Antibiotics found on the skin

A

defensins and cathelicidin

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59
Q

Th1

A

Attract macrophages and cytotoxic T cells

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60
Q

Th17

A

similar to Th1 but more powerful

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61
Q

Th2

A

stimulate macrophages to become alternatively activated

- recruits eosinophils and promotes IgE production

62
Q

Tfh (follicular)

A

migrate to B cell follicles in lymph nodes where they help B cells get activated

63
Q

Treg

A

make lymphokines that suppress activation of sibling T cells

64
Q

Tfh migrate from ___ into ___

A

deep cortex into follicles

65
Q

Type II immunopathology

A

autoimmunity

- cross react with self

66
Q

Type III immunopathology

A
  • someone make antibody against a soluble antigen
  • if antigen small, get trapped in basement membranes of capillaries
  • activate complement and inflammation occurs
67
Q

Type IV immunopathology

A

T cell mediated

68
Q

Chronic frustrated immune response

A

antigen not self, but something you can’t get rid of

EX: celiac and IBD

69
Q

Total WBC

A

4.5-10.5 x 10^9/ L

70
Q

Do young children have more lympohocytes or neutrophils

A

lymphocytes

71
Q

Arterioles and vein enter (exit) lymph nodes at the

A

hilum

72
Q

Lymph channels (afferent) enter at the

A

periphery

73
Q

efferent lymphatics

A

leave at hilum

74
Q

periarteriolar lymphatic sheath

A

surrounds central arteriole

mostly t cells

75
Q

Functional structure of Peyer patheces

A
  • specialized mucosal M cells (gatekeepers) injest proteins and transport to abluminal side
  • rich content of dendritic cells acquire antigens and carry to B follicles and T cells zones of peyer patch
76
Q

Germinal centers

A

tightly packed follicles

77
Q

Paracortec is located between the ___ and ___ and houses ___

A

follicles and medulla

T cells

78
Q

Papain + antibody =

A

Fab2

79
Q

Function of carbohydrate patch in antibody

A
  • pushes second constant domain of heavy chain out to interact with complement
80
Q

Two lymphatic circulations

A
  1. Lymph to blood (peripheral lymph nodes)

2. Blood to lymph (heart)

81
Q

Spleen has __ and ___ pulp

A

red and white

82
Q

Red pulp analogous to __ of lymph node

A

medulla

83
Q

Medulla of lymph node consists of ___ and ____

A

medullary cords and sinuses

84
Q

Medullary cords

A

Plasma cells

85
Q

Medullary sinus

A

reticular cells and macrophages

86
Q

Spleen is most important store of

A

monocytes

87
Q

Phagocytic cells have receptors (___) for altered Fc of ___ but not ___

A

FcR

IgG NOT IgM

88
Q

periarteriolar lymphatic sheath

A

surrounds central arteriole

mostly t cells

89
Q

Functional structure of Peyer patheces

A
  • specialized mucosal M cells (gatekeepers) injest proteins and transport to abluminal side
  • rich content of dendritic cells acquire antigens and carry to B follicles and T cells zones of peyer patch
90
Q

Conditions that must be met in order to activate a t or b cell

A
  • fit between receptor and antigen
  • several nearby receptors must be bound by antigen
  • accessory interactions*****
91
Q

Stimulated T cell becomes large and differentiated->

A

T lymphoblast

92
Q

An antigen that binds with low affinity may not ____, but if a aimilar antigen with higher affinity binds and activates a B cell:

A

activate a B cell

if activated, the product (secreted antibody) could combine with low affinity antigen and be inconvenient

93
Q

Function of carbohydrate patch in antibody

A
  • pushes second constant domain of heavy chain out to interact with complement
94
Q

How hypermutation works

A

Activation induced deaminase (AID) converts random cytosine to uracil.

  • Uracil removed by uracil-DNA glycosylase
  • DNA polymerase fills in gaps with single base substitution mutations
95
Q

Isotype

A

small AA differences in H chain C regions (contain subclasses)

96
Q

Idiotype

A

antibody’s unique set of CDRs considered as an antigen

97
Q

IgG conc in erum

A

1000 mg/dL

98
Q

1 dL = __ mL

A

100 ml

99
Q

IgA conc in serum

A

200 mg/ dl

100
Q

IgM con in serum

A

100 mg/dL

101
Q

Phagocytic cells have receptors (___) for altered Fc of ___ but not ___

A

FcR

IgG NOT IgM

102
Q

Soluble antigenic determinants are referred to as

A

Haptens

103
Q

Inhibitor of complement activity

A

C1 esterase inhibitor (C1-inh)

104
Q

Thymus is a __ component organ

A

two

  • lymphocytes whos precursers came from marrow
  • supporting structure or stroma
105
Q

Complement activation without antibody

A

alternative pathway

106
Q

An antigen that binds with low affinity may not ____, but if a aimilar antigen with higher affinity binds and activates a B cell:

A

activate a B cell

if activated, the product (secreted antibody) could combine with low affinity antigen and be inconvenient

107
Q

Hypermutability of the V(D)J unit allows for

A

Affinity maturation

108
Q

How hypermutation works

A

Activation induced deaminase (AID) converts random cytosine to uracil.

  • Uracil removed by uracil-DNA glycosylase
  • DNA polymerase fills in gaps with single base substitution mutations
109
Q

A single mature B cell begins by making

A

IgM and IgD

110
Q

During class switching what part of the antibody changes?

A

C region of H chain

111
Q

2 differentiated daughters of Hematopoietic stem cells

A

CLP and CMP

112
Q

Pro b cell

A

Mu chain in the cytoplasme

113
Q

Pre b cell

A

Cytoplasmic IgM NO SURFACE

114
Q

Immature B cell

A

Cytoplasmic and surface IgM but no surface IgD

115
Q

Mature

A

sIgM and sIgD

116
Q

When does a baby start making IgG

A

3-6 months postnatally

117
Q

In the thymus, there is a high concentration of ____ ligands that induce T cell differentiation

A

Notch

118
Q

Thymus is a __ component organ

A

two

  • lymphocytes whos precursers came from marrow
  • supporting structure or stroma
119
Q

People can completely reconstitute their T cell numbers and diversity up
to about ___ years of age

A

40

120
Q

Basophilic stippling

A

small dots on the periphery (ribosomes)

121
Q

Poikilocytosis

A

Variation in RBC shape

122
Q

Anisocytosis

A

Variation in RBC size

123
Q

Hyeprsegmented neutrophils are indicative of

A

megaloblastic anemia

124
Q

Dendritic cells are abundant where?

A

Skin, mucosa, GI tract

125
Q

Reticulocyte count is % of reticulocytes in ___ cells

A

1000

126
Q

2,3-BPG levels are high when (3)

A
  1. High altitude
  2. Hypoxia
  3. Homoglobin variance
127
Q

2,3-BPG levels are low when (4)

A
  1. Septic shock
  2. Transfusion of stored blood
  3. Methemoglobinemia
  4. CO poisoning
128
Q

Hemoglobin M inheritance

A

Autosomal dominant

129
Q

Hemoglobin M is a mutation in which globin chain? Resulting in what?

A

alpha or beta

Making F3 resistant to reduction

130
Q

Bone marrow consists of (5)

A
  1. Trabecular bone
  2. Osteoblasts and osteoclasts
  3. Sinusoids- leaky blood vessels
  4. Adipocytes
  5. Type 4 collagen fibers
131
Q

Blast forming unit->

A

colony forming unit

132
Q

How is iron overload diagnosed

A

Imaging studies

133
Q

Treatment of sickle cell with hydroxyurea leads to

A

50% reduction in acute pain events and acute chest syndrome

*** No evidence of reduction in chronic organ injury

134
Q

In the treatment of sickle cell anemia, there is an increased risk of hyperviscosity if

A

transfused to a hemoglobin > 10 g/dL

135
Q

Lytic complement

A

C9

136
Q

Marrow production is limited in that is can only increase by

A

6-8 fold

137
Q

Supravital stain

A

Allows identification of reticulocytes

138
Q

The last parameters to change when treating iron deficiency

A
  • FEP
  • RDW
  • MCV
139
Q

Endocrine gland most commonly affected in Cooleys anemia

A

Pituitary

lead to hypogonadotropic hypogonadism

140
Q

Reduction of CNS vasoocclusion in sickle cell disease

A

prophylactic blood transfusion

141
Q

Result of kidney tubule vasoocclusion in SC disease

A

inability to concentrate urine

142
Q

People with SC disease should expect to live into their

A

50s and 60s

143
Q

Why does fetal hemoglobin differ from HbA in oxygen affinity?

A

Fetal red cells have a higher oxygen affinity than adult red cells, primarily because hemoglobin F binds 2,3-BPG poorly, stabilizing the hemoglobin in the R state

144
Q

Treatment of unstable hemoglobins

A

folic acid

145
Q

committed precursor cell

A

BFU

146
Q

Bility of myeloid or lymphoid stem cell to self renew

A

can’t

147
Q

location of mast cells

A

tissue NOT blood

148
Q

Monocytes spend how long in the bone marrow

A

2-3 days

149
Q

Common areas for a pin crisis to develop

A

arms, legs, chest, abdomen

150
Q

Schistocyte->

A

Intravascular hemolysis

151
Q

Spherocytes->

A

Extravascular hemolysis

152
Q

Acute chest syndrome may be associated with

A

Kidney/liver failure

B&L random (6 decks)

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