Random Flashcards
1
Q
Ferroxidase present on the basolateral membrane
A
Hephaestin
2
Q
TrANSFERRIN SIZE AND WHERE IT IS PRODUCED
A
84 kd
lIVER
*** BINDING SPECIFICITY AND AFFINITY very high even at decreased pH
3
Q
Hepcidin size and where it is produced
A
25 aa
liver
4
Q
recycler of iron in the body
A
macrophage of the reticuloendothelium system
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
6
Q
Why do you continue iron treatment after hemoglobin is normal?
A
Need to increase ferritin stores
7
Q
Mutation that is the major cause of iron overload
A
HLA-H gene
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
9
Q
Each RBC contains how many hemoglobin?
A
approximately 280 million
10
Q
Size of hemoglobin
A
68 KD
11
Q
Mnemonic to remember basic shape of hemoglobin dissociation curve
A
10-10 30-30 60-90 40-75
12
Q
2,3 BPG
A
byproduct of anaerobic glycolytic pathway
stabilizes T conformation
13
Q
Normal concentration of 2,3 BPG
A
5 mmol/L
14
Q
Hemoglobin Gower I
A
Zeta2 Epsion2
15
Q
Hgb Gower II
A
alpha2 epsilon2
16
Q
Hgb Portland
A
Zeta2 gamma 2
17
Q
Hemolytic anemia forms
A
Heinz bodies
denatured hemoglobin
18
Q
Hgb Zurich
A
increases binding to CO
19
Q
Maintains iton in ferrous form within erythrocytes
A
NADPH methemoglobin reductase
20
Q
2 exposures that lead to methemoglobin
A
- Benzocaine (topical anesthetic)
2. well water contaminated with nitrates
21
Q
Normal CO levels
A
3%
22
Q
Smoker Co levels
A
10-15%
23
Q
Erythrocyte LS in circulation
A
120 days
24
Q
platelet LS in circulation
A
7-10 days
25
neutrophils ls in circulation
7 hour half life
26
Wright-stained reticulocyte
polychromatophilic
27
Pernicious anemia
autoimmune destruction of IF producing gastric parietal cells
28
Myloid to erythroid ratio megaloblastic anemias
erythroid predominance
29
Reaction involving B12 but not folate
synthesis of succinyl CoA from methymalonyl CoA
30
Schilling test
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
31
Target cells are characteristic of
thalassemias and HbE diseases
32
Inheritance of sickle cell
autosomal recessive
33
Things that cause oxidative stress to RBCs (4)
- sulfa drugs
- antimalarials
- infections
- fava beans
34
4 clinical manifestations of sickle cell anemia
1. increased retic count
2. increased WBC and platelet count
3. Increased RDW
4. increased unconjugated bilirubin, LDH, and AST
35
Most common chronic cause of death in adults with sickle cell disease
PAH
36
Most acute cause of death in sickle cell diseasse
Acute chest syndrome
37
bilirubin is ___ soluble
lipid
38
More water soluble form of bilirubin
urobiligen
39
Fate of urobiliogen
enterohepatic circulation or excreted by the kidney into the urine
40
Direct antiglobin test (DAT) evaluates the presence of (3)
IgG, C3d, or C4d
41
Inheritance of hereditary spherocytosis
most are autosomal dominant
| 25% are autosomal recessive
42
3 vaccinations pre-splenectomy
- H. influenza b
- S. pnemoniae
- meningococcus
43
After splenectomy
- prophylactic antibiotics (penicillin)
44
G-6-PD provides resistence to
plasmodium vivax
45
With oxidant stress, denatured hemoglobin does what?
attaches to the membrane and damages spectrin
46
Pyruvate kinase converts ____ to ____
phosphoenolpyruvate to pyruvate
47
In PK deficiency, the decrease in conversion of phosphoenolpyruvate to pyruvate causes (5)
decreased ATP and increased 2,3- BPG, loss of membrane plasticity, increase in rigidity, destruction in spleen
48
Things that cause oxidative stress to RBCs (4)
- sulfa drugs
- antimalarials
- infections
- fava beans
49
Treg
make lymphokines that suppress activation of sibling T cells
50
Cold antibody autoimmune hemolytic anemia ___vascular hemolysis
intravascular
51
Warm antibodies
IgG
52
Warm antibodies ____vascular hemolysis
extravascular
53
Steps of cold antibody autoimmune hemolytic anemia
- 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
54
Chronic frustrated immune response
antigen not self, but something you can't get rid of
| EX: celiac and IBD
55
Total WBC
4.5-10.5 x 10^9/ L
56
3 vaccinations pre-splenectomy
- H. influenza b
- S. pnemoniae
- meningococcus
57
After splenectomy
- prophylactic antibiotics (penicillin)
58
Antibiotics found on the skin
defensins and cathelicidin
59
Th1
Attract macrophages and cytotoxic T cells
60
Th17
similar to Th1 but more powerful
61
Th2
stimulate macrophages to become alternatively activated
| - recruits eosinophils and promotes IgE production
62
Tfh (follicular)
migrate to B cell follicles in lymph nodes where they help B cells get activated
63
Treg
make lymphokines that suppress activation of sibling T cells
64
Tfh migrate from ___ into ___
deep cortex into follicles
65
Type II immunopathology
autoimmunity
| - cross react with self
66
Type III immunopathology
- someone make antibody against a soluble antigen
- if antigen small, get trapped in basement membranes of capillaries
- activate complement and inflammation occurs
67
Type IV immunopathology
T cell mediated
68
Chronic frustrated immune response
antigen not self, but something you can't get rid of
| EX: celiac and IBD
69
Total WBC
4.5-10.5 x 10^9/ L
70
Do young children have more lympohocytes or neutrophils
lymphocytes
71
Arterioles and vein enter (exit) lymph nodes at the
hilum
72
Lymph channels (afferent) enter at the
periphery
73
efferent lymphatics
leave at hilum
74
periarteriolar lymphatic sheath
surrounds central arteriole
| mostly t cells
75
Functional structure of Peyer patheces
- 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
Germinal centers
tightly packed follicles
77
Paracortec is located between the ___ and ___ and houses ___
follicles and medulla
| T cells
78
Papain + antibody =
Fab2
79
Function of carbohydrate patch in antibody
- pushes second constant domain of heavy chain out to interact with complement
80
Two lymphatic circulations
1. Lymph to blood (peripheral lymph nodes)
| 2. Blood to lymph (heart)
81
Spleen has __ and ___ pulp
red and white
82
Red pulp analogous to __ of lymph node
medulla
83
Medulla of lymph node consists of ___ and ____
medullary cords and sinuses
84
Medullary cords
Plasma cells
85
Medullary sinus
reticular cells and macrophages
86
Spleen is most important store of
monocytes
87
Phagocytic cells have receptors (___) for altered Fc of ___ but not ___
FcR
| IgG NOT IgM
88
periarteriolar lymphatic sheath
surrounds central arteriole
| mostly t cells
89
Functional structure of Peyer patheces
- 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
Conditions that must be met in order to activate a t or b cell
- fit between receptor and antigen
- several nearby receptors must be bound by antigen
- accessory interactions*****
91
Stimulated T cell becomes large and differentiated->
T lymphoblast
92
An antigen that binds with low affinity may not ____, but if a aimilar antigen with higher affinity binds and activates a B cell:
activate a B cell
| if activated, the product (secreted antibody) could combine with low affinity antigen and be inconvenient
93
Function of carbohydrate patch in antibody
- pushes second constant domain of heavy chain out to interact with complement
94
How hypermutation works
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
Isotype
small AA differences in H chain C regions (contain subclasses)
96
Idiotype
antibody's unique set of CDRs considered as an antigen
97
IgG conc in erum
1000 mg/dL
98
1 dL = __ mL
100 ml
99
IgA conc in serum
200 mg/ dl
100
IgM con in serum
100 mg/dL
101
Phagocytic cells have receptors (___) for altered Fc of ___ but not ___
FcR
| IgG NOT IgM
102
Soluble antigenic determinants are referred to as
Haptens
103
Inhibitor of complement activity
C1 esterase inhibitor (C1-inh)
104
Thymus is a __ component organ
two
- lymphocytes whos precursers came from marrow
- supporting structure or stroma
105
Complement activation without antibody
alternative pathway
106
An antigen that binds with low affinity may not ____, but if a aimilar antigen with higher affinity binds and activates a B cell:
activate a B cell
| if activated, the product (secreted antibody) could combine with low affinity antigen and be inconvenient
107
Hypermutability of the V(D)J unit allows for
Affinity maturation
108
How hypermutation works
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
A single mature B cell begins by making
IgM and IgD
110
During class switching what part of the antibody changes?
C region of H chain
111
2 differentiated daughters of Hematopoietic stem cells
CLP and CMP
112
Pro b cell
Mu chain in the cytoplasme
113
Pre b cell
Cytoplasmic IgM NO SURFACE
114
Immature B cell
Cytoplasmic and surface IgM but no surface IgD
115
Mature
sIgM and sIgD
116
When does a baby start making IgG
3-6 months postnatally
117
In the thymus, there is a high concentration of ____ ligands that induce T cell differentiation
Notch
118
Thymus is a __ component organ
two
- lymphocytes whos precursers came from marrow
- supporting structure or stroma
119
People can completely reconstitute their T cell numbers and diversity up
to about ___ years of age
40
120
Basophilic stippling
small dots on the periphery (ribosomes)
121
Poikilocytosis
Variation in RBC shape
122
Anisocytosis
Variation in RBC size
123
Hyeprsegmented neutrophils are indicative of
megaloblastic anemia
124
Dendritic cells are abundant where?
Skin, mucosa, GI tract
125
Reticulocyte count is % of reticulocytes in ___ cells
1000
126
2,3-BPG levels are high when (3)
1. High altitude
2. Hypoxia
3. Homoglobin variance
127
2,3-BPG levels are low when (4)
1. Septic shock
2. Transfusion of stored blood
3. Methemoglobinemia
4. CO poisoning
128
Hemoglobin M inheritance
Autosomal dominant
129
Hemoglobin M is a mutation in which globin chain? Resulting in what?
alpha or beta
| Making F3 resistant to reduction
130
Bone marrow consists of (5)
1. Trabecular bone
2. Osteoblasts and osteoclasts
3. Sinusoids- leaky blood vessels
4. Adipocytes
5. Type 4 collagen fibers
131
Blast forming unit->
colony forming unit
132
How is iron overload diagnosed
Imaging studies
133
Treatment of sickle cell with hydroxyurea leads to
50% reduction in acute pain events and acute chest syndrome
*** No evidence of reduction in chronic organ injury
134
In the treatment of sickle cell anemia, there is an increased risk of hyperviscosity if
transfused to a hemoglobin > 10 g/dL
135
Lytic complement
C9
136
Marrow production is limited in that is can only increase by
6-8 fold
137
Supravital stain
Allows identification of reticulocytes
138
The last parameters to change when treating iron deficiency
- FEP
- RDW
- MCV
139
Endocrine gland most commonly affected in Cooleys anemia
Pituitary
| lead to hypogonadotropic hypogonadism
140
Reduction of CNS vasoocclusion in sickle cell disease
prophylactic blood transfusion
141
Result of kidney tubule vasoocclusion in SC disease
inability to concentrate urine
142
People with SC disease should expect to live into their
50s and 60s
143
Why does fetal hemoglobin differ from HbA in oxygen affinity?
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
Treatment of unstable hemoglobins
folic acid
145
committed precursor cell
BFU
146
Bility of myeloid or lymphoid stem cell to self renew
can't
147
location of mast cells
tissue NOT blood
148
Monocytes spend how long in the bone marrow
2-3 days
149
Common areas for a pin crisis to develop
arms, legs, chest, abdomen
150
Schistocyte->
Intravascular hemolysis
151
Spherocytes->
Extravascular hemolysis
152
Acute chest syndrome may be associated with
Kidney/liver failure
