Exam 1 Flashcards
1
Q
Positive cooperativity
A
oxgen binding to hb alters confirmation to increase affinity for other oxygen molecules
2
Q
Acute chest syndrome associated complications
A
kidney and liver failure
3
Q
How does temp influence saturation curve?
A
shifts to the right
4
Q
Vitamin B12 absorption
A
in stomach with Intrinsic Factors, gets absorbed in ileum and is released from IF
5
Q
Thrombopoetin
A
differentiates and matures patelets into periphery, originates from other cells and small component of kidney
6
Q
Treatment of Chronic infection/inflammation/malignancy
A
treatment of underlying disease to decrease cytokines and interluekins
7
Q
Redicualte in circulation
A
1-2 days
8
Q
Idiotype
A
difference in CDR due to specificity.
9
Q
Pro-Bcell
A
just heavy chain with VDJu
10
Q
Calculated Hematocrt
A
RBC * MCV
11
Q
Alpha globin in DNA
A
on CH16, 2 paternal and 2 maternal; Zeta during development
12
Q
post-capillary venules in lymph node
A
high-culoidal to react to lymphocytes
13
Q
too few monocytes
A
monocytopenia
14
Q
path of lymphocyte
A
bind to high cuboidal cell, pass between into the node to lymph, to lymph channels, to thoracic duct to venou blood and back
15
Q
IgD
A
long hinge region, 5 mg/dl; 180,000 D
16
Q
Hb Portald 1
A
Z2Y2
17
Q
RDW calculation
A
standard deviaiton of values of MCV
18
Q
TLR
A
bind to initiate inflammatioon cascade and ultimately activate NFkB
19
Q
Allotypic exclusion
A
like X inactivation of antibodies, recombination can be done if initial antibody is unsuccessful
20
Q
Hemolytic anemais
A
Hereditary spherocytosis, enzyme disorders, autoimmune hemolytic anemia
21
Q
Fe Deficient - Concentrations
A
99% a1; 2% A2;
22
Q
MCV
A
Mean Corpsular Volume, average volume of RBC
23
Q
Spectrin, ankryn, band 3 alll..
A
lead to destabalization of lipid bilayer and microspherocytes –> deformability
24
Q
How does temperature affect oxygen affinity
A
Higher temperature unloads oxygen easier
25
Retic count in hemolysis
increases
26
Immunogen
antigen that generates immune response
27
symptoms of G6PD deficiency
acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies
28
Direct Antiglobin testing
IgG, C3d, C4dl tests directly in patient blood
29
How does dropping the pH shift the Saturation curve
to the right, -lower the affinity of oxygen
30
monocytes
immature macorphages or dendritic cells comprise 2-8% of WBC
31
Hb Poole
mutation in gamma chain, phycial unstable variant; infants have hemolytic anema, but it resolves with time.
32
Characteristics of monopoesis
delicate chormatin pattern, gains vaculoles during maturation, abundance of cyto
33
Maturation of B lymphoblast
to B lymphocyte to Plasma cell with lots of golig and ER
34
what factors influence oxgygen dissocation?
pH, temperature, 2,3-BPG
35
Papin
reduces disulfide bonds to create 2 Fab and 1 Fc
36
Gestational Hemoglobins
Gower ! And II, and Portland I and II
37
IgE
extra long C4, 0.2 mg/dl; 190,000 D
38
HbE
Beta 26 Glu --\> Lys; SE asia
39
Direct Current Technology
blood passes through electrodes and creates resistance in proprotional to cell size. RBC and platlets could be measured simultaneously.
40
Hypocellular marrow is called\_
autoimmmune attack, viral attack
41
What causes hypercellular marrow?
hypoxia, increase in HGFs
42
Taut
T hb in low oxygen situations. Four unoccupied sites, binding affinity is low.
43
Where does megakaryopoiesis take place?
in bone marrow along vascular sinuses and integrates into lumen of vessels and release plasma into bloodstream
44
post-capillary venules in lymph node
high-culoidal to react to lymphocytes
45
Germinal center
sites of intense lymphocyte division (b cell). Increase cytoplasm compared to nucleus
46
Fc
botton region of heavy chains with carbohydrate to prevent collapse
47
VCAM1
increased in SCD, increased cell adhesion to endoethelium
48
Maturation of B lymphocyte
to B lymphoblast to Plasma cell with lots of golig and ER
49
Where does Hb F remain high?
Infants with mothers who have diabetes, hemolytic anemia, bone marrow diseases
50
Alpha Thal concentations
97 A1; 3% Ab2;
51
mature B cell
sIgM and sIgG with same specificity
52
Too many eosinophils
eosinophilia
53
Monopoesis
Requires M-CSF; monoblast --\> promonocyte --\> monocyte
54
Trends in Eosinophils
always 0-6%
55
lifespan of sickled cell
56
SC - NBS
FSC
57
Cholelithiasis
gallstones due to increased bilirubin, --\> obstruction
58
Retic count - SCD with B+ Thal
3-5%
59
Low Affinity Hb variants Symptoms
mild anemia, cyanosis
60
What prevents infection in SCD?
penicillin porphylaxis
61
alpha -thal NBS
Fbarts
62
primary RNA transcript in antibody genes
made through Cu and Cdelta
63
Decreased RBC survival in thalassemia
increased retic count, intreacsed intracellular RBC contnets, splenomegaly, bilirubin gallstones, anemia
64
MCHC calculation
hb/hct
65
How does mRNA for antibody form?
splicesome splices out primary RNA to make VDJ u or VDJdelta
66
monocytes
immature macorphages or dendritic cells comprise 2-8% of WBC
67
NK cells
differentiated B cell, with immunity and survelliance
68
TNF
stimulated by malignancies and sepsis, to decrease iron and therefore erythropoeitin and inhibit RBC production
69
what converst bilirubin to glucuronic acid
CYP450 in liver
70
Splenic Function
clearance of intravascualr particles; adaptive immune resonse: origin of igM
71
what joins first in VDJ sequence
DJ
72
Beta Globin in DNA
CH11, Epsilon during development, one copy from each parent
73
Too many neutrophils
neutrophilia
74
paracortex in lyph node
not as dense as cortex, houses the T cells
75
C5A
chemotactic agent to attract bacteria in complement pathway
76
reticulcoytes time in marrow
3-4 days
77
MCH
mean corpsular hemoglobin; weight of Hb of the average red cell
78
what joins first in VDJ sequence
DJ
79
clinical features of renal insufficiency
fatigue, pallor decreased exercise tolerance, dyspena, tachypena, anemia - unrepalted to kidney dysfunction
80
Opsonizing
opsonizationÿis a means of identifying the invading particle to the phagocyte; labled with igG and C3B to act as handles for phagocytosis
81
Infections with SCD is due to
due to spenid dysfunction and aplastic crisis
82
IgD
long hinge region, 5 mg/dl; 180,000 D
83
order these in order of sicking potenial - SS, SF, SC, SF
SF
84
Splenic Sequestration
blood flow into sinusoids is occluded and unable to flow out. Splenomegaly
85
Complications with hereditary spherocytosis
aplastic crisis and cholelithiasis
86
IgA
dimer with J chain and secretory component, 400,000D
200 mg/dl
87
IgG Serum
1000 mg/dl; lightest at 150,000 D
88
Granulopoiesis
creation of eosinophil, basophils, and neutrophils
89
calculated components of a CBC
Hct, MCV, mean capsular hb, mean capsular hb conc, RDW, absolute leukocytes
90
Mononulcear cells make
monocytes and lymphocytes
91
2,3 - BPG
byrpoduct of anaerobic glycolysys. During increased O2 use, hypoxia, chronic anemia, more is produced because glycolysis increases
92
what factors increase sickling?
deoxygenation, potassium, dehydration, Hb concentration
93
DAMPS
damage associated molecular patterns - sense internal damage of our own cells with TLR and attarct phagocytic cells
94
Complement pathway
completes antibody process by activating C3 to activates membrane attach complex
95
Immune complex
froms when antibodies bond to antigens and cross link due to divalnce at equal concetnraiton of antibody to antigen.
96
T Cell and B cell distribution in spleen
T cell surround arterioles and B cells surround them
97
Endothelial-1
vasoconstrictor, increases in SCD
98
Microcytic anemia types
Fe deficiency, thalassemia, lead poisoning
99
when does fetus make igG?
3 months old
100
Direct Antiglobin testing
IgG, C3d, C4dl tests directly in patient blood
101
how is a differential performed?
blod film (wdge, cover glass, spinner), air dry and fix with MeOH, wright-giemsa stain
102
Lab finding of AIHA
Increased Retic, increased bilirubin, hemoglobinuria, warm + Dat for igG or Cold + Dat for complement
103
paracortex in lyph node
not as dense as cortex, houses the T cells
104
Hb Chesapeak symptoms
hypoxia, generally well affected and don't need treatment
105
Hb Zurich
point muations to increase binding to CO
106
Eosinophils in Differential
Bi-lobed nucleus, large red granuals
107
igM
pentamer, extra long C4, S-S bonds and One J chain, 900,000D; 100 mgdl
108
seg
muliple lobes with chormatin band, small size, pink cyto
109
Too many basophils
basophilia
110
Absorption of Folate
in jejunum where it is hydrolyzed, reduced, methylated into methyltetrahydrofolate.
111
what is practicla reference interval in CBC?
mean +- 2 SD
112
IgM (classes)
immunization to activatio of complement, V=10, but binds to 2. vicsous, only ab made in fetus
113
Immunological Aging
as we age, our memory cells outnumber our na‹ve cells and cant respond to new antigens.
114
T Cell and B cell distribution in spleen
T cell surround arterioles and B cells surround them
115
Opsonizing
opsonizationÿis a means of identifying the invading particle to the phagocyte; labled with igG and C3B to act as handles for phagocytosis
116
Clonal Selection theory
cells make one antibody, choice is randome dependent on outside signals.
117
HBO
Arab; Beta 121 Glu --\> lys (basic); Africa, Middle east
118
Domains in antibodys
made of 7 Beta sheets for rigidity. The region sin between the sheets form the variable regions. Held together by disulfide bonds
119
Lymphocytes in differential
scant cyto with dense, round nuclus
120
Cyanosis
blue/grey color skin and mucus membrane; Too must deoxyhemoglobin 1.5 g/dl methemoglobin (8-12%); sulhemoglobin \>0.5 g/dl
121
Hb at birth
65-95% Hb F and 20% HbA
122
Warm
IgG, poor complement activity activates spenic macrophages and extravacualr hemolysis
123
when does fetus make igM?
3 months gestation
124
Basophils - immuno
acidic cells, release histamine, Mast cells in tissue; 0.5-1%
125
Fetal Hemoglobin
alpha2gamma2
126
How is bone marrow biopsy performed?
through ilica crest, suction to pull out reiculyn fibers; use a core to pull out a piece of marrow that contains bone and cells.
127
Leukocyte absolute count
Diff% \* WBC / 100
128
HbS
Beta 6 Glu --\> Val (acid to hydrophobic); africa, india, med
129
Endocrine disorders lab findings
mormochronic and cytic, decreased retic count
130
AS - NBS
FAS
131
MCV calculation
Hct/RBC count
132
Humoral
antibody mediated immunity
133
What contributes to antibody diversity
recombination, exonucleases, terminal deoxynucleotidyl transferase added random NT to N region
134
Protein Calorie Malnutrition
anemia due to lack of protein and calories
135
what is %sat at 60 mmHg
90%
136
Normocytic anemia types
anemia of chronic disease, sickle cell
137
Symptoms of AIHA
acute or chronic anemia, pallor, jaundice, dark urine, splenomegaly
138
Splenic InfarctioN
insufficient bloodflow to spleen (usually by age 4 in SCD)
139
Iron Deficienty lab finidngs
decreased MCV, RBC, MCV/RBC \>13, incrase RDW, Decreased ferritin, normal electrophorysis, response to Fe
140
Immune complex
froms when antibodies bond to antigens and cross link due to divalnce at equal concetnraiton of antibody to antigen.
141
EB+
FEA
142
G-CSF
helps early blast stage differenitiating into myeloid lineage as opposed to lmphoid.
143
C5A
chemotactic agent to attract bacteria in complement pathway
144
Hematocrit
erythrocote volume/whole blood volume - centrifugation
145
pathway for malignancis/sepsis in anemia
production of TNF and Interfereon Beta
146
what does manual differential add?
morphology of RBC, WBC, platelets, abnormalities in peripheral blood, relative or absolute quantification of WBC populations
147
Hemopexin
binds to free heme from methemogobin to be processed in liver to make bilirubin
148
Fab
top part of one heavy and one light chain
149
Methemoglobin - how do you get it?
acquired or genetic
150
what kind of infection is caused by SCD
encapsulated bacteremia - strep, hemophilus influenza, meningococcus
151
Affinity Maturation
with each replication CDR varies, if antibody encounters antigen it stays in the game.
152
igM
pentamer, extra long C4, S-S bonds and One J chain, 900,000D; 100 mgdl
153
Hemoglobin amount - SCD trait
14-16 mg/dl
154
Maturation of T lymphocyte
T lymphoblast
155
How long does MVC take to get back to normal in B12/Folate defidiency
months
156
How long does Hb take to get back to normal in B12/Folate defidiency
1-2 weeks
157
when does fetus make igM?
3 months gestation
158
Sickel Mephropathy
10-15%; hyperfiltration and enlarged glomeruli, porteinuria
159
what can you measure with flow cytometry?
Reticulocyte count, nucleated RBC types, platelet count
160
Treatment of hereditary spherocytosis
supportive care, folate, splenectomy
161
Epitope
region of antigen that interacts with CDR, discontinous or continous, falt, also called antigenic determinants. Interacts with CDR non-covalenly.
162
what globin chain makes antibodies
gamma (called immunoglobulin
163
Rag recombinase
enzyme responsible for looping out random parts of DNA to greate VDJ recombination
164
Symptoms of AIHA
acute or chronic anemia, pallor, jaundice, dark urine, splenomegaly
165
medulla role lymph node
filter
166
measuring MCV
mean height of the voltage pulse during RBC count
167
hemoglobincyanide method
combine bood with potassium cyanide and potassium ferricyanide, product absorbs light at 540 nm. Can detect how much Hb based on absorbance.
168
macrocytic anemia types
vit B12 and volate deficience
169
HbH
B4
170
Retic count - HbSC
3-5%
171
HbEE
FE
172
Tolerogen
antigen that does not ellicent and immune response
173
Less severe sickle cell types
SB+, SC, trait (no anemia)
174
how to limit iron loading
erythroectapheresis and phlebotomy prior to transfusion
175
Mitotic pool of granulopoiesis
4-5 cell divisions, where they enter the storage pool with no more cell division
176
what globin chain makes antibodies
gamma (called immunoglobulin
177
Retic count - SC Trait
1-2%
178
Pulmonary hypertension
increased pressure on pulmonary arteries, most often due to sickle lung disease
179
What type of bilirubin is expelled in feces?
fecal urobilinogen
180
Lab findings of lead intox
mild/mod anemia, microcytosis, mild hypochormia, basophilic stippling, increase zinc protoporphyrin.
181
Sickle lung disease
25-40% of SCD; preogressive obliteration of pulmonary vasculature; leading cuase of death in adults with SCD
182
Immunological Aging
as we age, our memory cells outnumber our na‹ve cells and cant respond to new antigens.
183
Alternative complement pathway
cell wall structures in microbes activate C3 directly in absense of antibody (innate)
184
HbC
Beta 6 Glu --\> Lys (acid to base); west africa
185
RDW
Red blood cell distribution width
186
order of antibody sequence for DNA
VDJ
187
Hb A2
alpha 2 delta 2
188
iron in RBC transfusion
1ml has 1 mg Fe; 3-4 g Fe/yr
189
Idiotype
difference in CDR due to specificity.
190
Bilirubin in extravascualr hemolysis
porphyrin ring forms bilirubin, which is conjugated to glucoronic acid in liver and then in bowel is converted to urobilinogen
191
Complement pathway
completes antibody process by activating C3 to activates membrane attach complex
192
Pathophysiology of thalassemia
Imbalance alpha and beta, excess globin participate, ineffecient erythropoesis
193
Monocytes in differential
largets, irregulat nulcues, fine granuals, vacuolated cytoplasm
194
Complications with hereditary spherocytosis
aplastic crisis and cholelithiasis
195
Eosinophils - immuno
basic cells, bind to major basic proein, kill parasites. 1-4%
196
Sickle Dex
hemolyze blood with reducing agent, and release Hb. Sickle is less soluble. Can detect SBD, but not type of disease
197
Hb Portland 2
Z2 B2
198
half life of igG?
3 weeks
199
What is P50 or hb 50% saturated?
27 mmHg
200
Hydroxyurea and bytyrate
fetal Hb induction; reduces sickle hb polymerization, decreases WBC count
201
Granulopoiesis starts with\_
myloblast to promeylocytes that splits into the three idfferent lineages as bands and then seg.
202
Cathelicidin
bind to negative charged pathogens and bore holes - located on skin/mucosa
203
Allotype
differences in antibody due to mom or dad, in non-CDR region
204
Beta thal trait concentrations
94 A1; 4% A2; 2% HbF
205
EB0
FE
206
Anemia Concentration - normal
97% A1 and 3$% A2;
207
SB+ = NBS
FSA
208
Microcytic anemia types
Fe deficiency, thalassemia, lead poisoning
209
Heptoglobin
plasma proin that binds to HB dimer that is created in intravascular hemolysis; removed from circulation in liver
210
order of antibody sequence for DNA
VDJ
211
erythroceytapheresis
erytrocytes separate from whole blood
212
Neutrphils in Differential
Acidic cytoplasm, 2-5 distinct lobular nuclues
213
Lymphocytes - immuno
T and b cells; prominent WBC in children; 20-40%
214
Vaso-occlusive Pain
acute complication of SCD; pain in muslces and back and require opiates and antiinflammatory.
215
when are transfusions used?
only when severity has potential for cardiovascualr decompensation
216
half life of igG?
3 weeks
217
50-60% methemoglobin
stupor, bradycardia, respiratory depression, convulsions, acidosis
218
is normal good?
in certain diseases tests are expected to be abnormal. Failure to chagne is bodys inability to respond approprioately.
219
Hemoglobin amount - SCD
6-9 mg/dl
220
Causes of V12 deficiency
pernicicious anemia - autoimmune
221
Biochemical test of Folate Deficiency
decreased serum folate.
222
RBC range for Direct Current
35-250 fl
223
Schlling Test
ingetion of radiolabeled Vit B12, it gets absorbed into blood stream, give flushing dose of normal V12 IM at 2 hours; Kidney normally excretes 3-35% of absorbed radioactive in 24 hours
224
lab finding for hereditary spherocytosis
increased retic count, decrased MCV, spherocytes
225
Where does curve lean towards in gel test for Ab and AgWhere does curve lean towards in gel test for Ab and Ag
Ab (they are larger)
226
What malignant diseases cause anemia
carcinoma, hodgkins disease
227
what causes increase iron absorption
ineffecte RBC production, low hepcidin, mutations in HFE
228
stroke in SCD
cellular adhesion, loss of vasoregulation by increased endothelial 1 release, and decreased NO.
229
SCD concentrations
2-30% HbF 70-98% HbS
230
Sickle Cell Trait Risks
kidney complications: microscopic hematuria, renal papillary necrosis, isosthenia, chronic kidney disease; blood clot risk; splenic infarction, rhadomyolysis
231
Cold
IgM (some IgG) bind in extremitie and return in warmth to acivate complement and intravascualr hemolysis
232
Polymorphonuclear cells
eosinophils, basophils, neutrophils
233
use of myoglobin
oxygen storage in muscle
234
Alpha thalassemia lab findings
decrease MCV, increased RBC, MCV/RBC
235
Class switching
V domain stays the same, but splicing occurs at DNA level to cut out other constant regions. Cannot go back to the ones cut out
236
HbH thalassmeia
3 deletions; moderate anemia, makes B4 to compensate, transfusion depent on stress
237
Methemoglobin symptoms
asymptomatic, but low sat; dark blood when mixed with air.
238
How does increasing pH shift the saturation curve?
Shift to the left - increased affinity
239
Hydroxyurea and bytyrate
inductio of HbF
240
primary RNA transcript in antibody genes
made through Cu and Cdelta
241
PAMPs
pathagen associated molecular patterns, recognize forein structures through interaction with PRR
242
IgA
dimer with J chain and secretory component, 400,000D
243
HB SB0 concentrations
3% A2; HbF 2-30; S 70-98
244
macrocytic anemia types
vit B12 and volate deficience
245
PRR
pathogen recognition receptors are on surface or inner membrans (TLR)
246
symptoms for PK deficiency
variable anemia, hemolysis, splenomegaly, gallstone, aplastic crisis
247
DAMPS
damage associated molecular patterns - sense internal damage of our own cells with TLR and attarct phagocytic cells
248
Beta thal and Fe deficient concentrations
96 A1; 3% A2; 1% HbF
249
gallstones due to increased bilirubin, --\> obstruction
decreases pyruvate formation and decreases ATP production; loss of membrane plascticity and increased rigidity.
250
Humoral
antibody mediated immunity
251
Types of anemia in Hereditary Spherocytosis
5% sever; 60-75% moderate; 20% mild
252
methemalbumin in hemolytic anemia
increases
253
Lectin pathway
complement pathway; mannose bidning protein binds to bacterial structures to activate Complement pathway MBP- 4-2-3-5-6-7-8-9
254
Sideroblastic anemia clinical/lab
variable anemia, hypochromia, microcytosis, accumulation of iron in mito
255
G-6PD Def mechanism
deecrased production of NADH, reduction in glutathione, and protecton from hydrogen peroxide.
256
medulla role lymph node
filter
257
clinical features of Chornic infection, inflammation, malignancy
dependent on underlying disease
258
Current mothod of measuring Hb
lyse RBC and WBC, alter globin and oxidize heme, sodium lauryl sulphate binds to form colored complex and measure the absorbance.
259
how does chronic inflammation/infection cause anemia?
stimulates IL1 and interferon gamma
260
Neutrophil killing
1) phagocytosis or 2) degranulation (spews out granules into enironment to cause necrosis and inflammation)
261
Gower 2
alpha2E2
262
Warm
IgG, poor complement activity activates spenic macrophages and extravacualr hemolysis
263
Risks in transfusion
infection, allo-immunization; iron overload
264
Immunogen
antigen that generates immune response
265
Iron loss per day
1mg/day; fixed; sloughed mucosa, meses
266
iron overload toxicity
ears, hearing, bone, liver, kidney
267
Trend of neutrophils over lifetime?
45-50% at birth until 2-12 years old increase to 40-75%
268
Treatmetn of acute chest syndrome
transfusion to maintain 9-10 mg/dl, induction of HbF, bone marrow transplant
269
what happens in thalassemia
Anemai, bone marrow expansion, extramedullary hematopoesis
270
how does pH affect O2 dissociation?
Oxygen is held tightly in basic conditions and released in acidic
271
Immature B cell
cIgM and surface IgM; clonal deletion
272
heptoserum levels in hemolysis anemia
decrases
273
Hereditary Spherocytosis
most often due to spectrin deficiency, causes anemia jaundice, splenomegaly. Microspherocyte and osmotic fragility
274
Monopoesis circulation
marginal pool for 20 days and can go into tissue to become macrophages; circulation is short lived.
275
Mononulcear cells make
monocytes and lymphocytes
276
Retinopathy
causes neovascularization (sea fan formation) and venular occlusion (sea fan infarction); redintal detachment, hemorrahge, and blindness
277
Too few neutrophils
neutropenia
278
How long does blood count take to get back to normal in B12/Folate defidiency
8 weeks
279
Hemoglobin amount - Sickle B0 Thal
6-9 mg/dl
280
Fc
botton region of heavy chains with carbohydrate to prevent collapse
281
Brend of Bands over lifetime?
always 0-9%
282
Allotype
differences in antibody due to mom or dad, in non-CDR region
283
Transcoalbumin BP II
binds to Vitamin B12 after ileal abosprtion and promotes liver sotrage or transfer to tissue (bone marrow).
284
oxidation of Hb\_ from G6PD deficience
denatures Hb so it attaches to membrane to create heinz bodies, trapping/deformability and extravascualr hemolysis
285
Clinical manifestations of thalassmeia
Underproduction of normal Hb and decreaesd RBC survival
286
Eosinophils - immuno
basic cells, bind to major basic proein, kill parasites. 1-4%
287
Lead intoxification and anemia
lead inhbits protoporphyrin ring syntehsis and iron from forming hemoglobin
288
G-6-PD deficiency
X-linked recessive; prevalent in tripical eastern hemisphere, selective resistance to malaria
289
Peyer Patch
lymph node I n gut that underlies mucosa of SI; located by goblet cells. Phacogytose proteins and particles for luminal transport. Determine if food or threat
290
IgE
extra long C4, 0.2 mg/dl; 190,000 D
291
IgE (class)
binds to mast cells and eosinophils, triggers histamine release for allergic reactions; parasite killer
292
hematologic changes in folate and B12 deficiency?
megablastic, macrocytosis, decreased Retic index, hypersegmented neutrofils, increased biliruin and LDH
293
Extravascualr hemolysis
done by macrophages in RE system of spleen
294
Dendritic cell mechanism
cytokine,chemokine, stimulate immature DC to phagocytose everything, activate DC enters lymphatic cystem to nearly lymph node, puts digested pathogen on surface as antigen presentingmolecule for T and B cell --\> activates adaptive response.
295
Partial pressure in tusse
40 mmHg
296
why is aplastic crisis dangerous for hereditary spherocytosis?
severe drop in hct due to shortened RBC lifespan
297
lymph flow in lymph node
entry in periphery, through subscapular sinus through middle of node and out of hilum
298
Clonal delection
if IgM on surfae encounters self, it initiates apoptosis. If it doesn't encounter antigen in 24 horus, unlikely to antibody towards self and can surive
299
Flow Cytometry
lysis regaent performates membranes but leaves cells intact, label DNA and RNA anre fluorescently labeled, scattergram according to fluroescence and internal struture. Strongest fluroescence is immagure with high NA content.
300
why is aplastic crisis dangerous for hereditary spherocytosis?
severe drop in hct due to shortened RBC lifespan
301
Hemolysis in SCD
creates more free Hb and inhibits NO release and vasodilation; also increases erythroid arginase to degrade arginine and precursor to NO.
302
Treatment of Beta thal
transfusion to maintain 9-10 mg/dl, induction of HbF, bone marrow transplant
303
HbA2
2% of normal HbA; functions like HbA1, but more heat stable and slightly higher oxygen affinity.
304
Lymphocytes - immuno
T and b cells; prominent WBC in children; 20-40%
305
C1-esterase inhibitor
regualtes the actiation of C3 so complement pathway is not always activated
306
Relaxed hb
loosey goosy hb, as binding to oxygen increases salt bonds are brokena and affinity increases
307
Band
more than 1/2 dimeter of nuclear indentation
308
INF gamma
inhibits erythroid proliferation and decrease RBC production due to chronic infection and inflammation
309
G-6PD Def mechanism
deecrased production of NADH, reduction in glutathione, and protecton from hydrogen peroxide.
310
Folate and Vitamin B12 do\_
support proliferation and maturation of RBCs, methionine sysntehsis, purine and pyrmidine biosynthesis, production of thymodylate for DNA synthesis
311
oxidation of Hb\_ from G6PD deficience
denatures Hb so it attaches to membrane to create heinz bodies, trapping/deformability and extravascualr hemolysis
312
what happens with stress to reticulocytes
early release by 5-7 days
313
Trends of Lymphocytes over lifetyme
1-3 days 15-30%; 4-14 days 4-18%; 15 days to 2-12 years 2-14%; 13 + 2-11%
314
Pro-Bcell
just heavy chain with VDJu
315
IgM (classes)
immunization to activatio of complement, V=10, but binds to 2. vicsous, only ab made in fetus
316
Gower 1
Z1 E2
317
Hb Koln
mutation in beta chain, physican unstable varian. Has increased oxygen affinity.
318
Hb SC concentrations
319
Fab
top part of one heavy and one light chain
320
Methods for SCD testing
Sickle Dex, HB separation, isoelectric focusing, HPLC
321
Chronic inflammatory disease cause anemia
rheumatoid arthritis, systemic lupus erythematosis, rheumatic fever
322
path of lymphocyte
bind to high cuboidal cell, pass between into the node to lymph, to lymph channels, to thoracic duct to venou blood and back
323
IgA (class)
made by plasma cell in mucus membrane; secretory component, small amoutn of monomer in IgA plasma
324
isosthenuria
urinary concentratin defect
325
Hemolytic anemais
Hereditary spherocytosis, enzyme disorders, autoimmune hemolytic anemia
326
adverse effects of iron overload
hepatic fibrosis and cirrhosis, endocrinopathies, cardiomyothpathy and sudden death
327
Treatment of folate deficiency
1mg/day orally or parenterally
328
How does 2,3- BPG concentration affect oxygen affinity
stabalizes T conformation and leads to decreased oxygen affinity
329
Trends of Monocytes overtime
1-3 days 15-30%; 4-14 days 4-18%; 15 days to 2-12 years 2-14%; 13 + 1-3 days 5-20%; 4-14 days 4-18%; 15days to 2-12 years 2-14%; 13+ 2-11%
330
when is EPO used?
when there is an absolute deficiency or decrease of EPO out of proportion to hct
331
IgA (class)
made by plasma cell in mucus membrane; secretory component, small amoutn of monomer in IgA plasma
332
Germinal center
sites of intense lymphocyte division (b cell). Increase cytoplasm compared to nucleus
333
Hydroxyurea and complications
50% reduction in acute pain events, no evidence for chronic organ injury
334
Tolerogen
antigen that does not ellicent and immune response
335
how much can bone marrow production increase
6-8 fold
336
Trends in basophils
always 0-2%
337
Mekagaryopoiesis
Thrombopoesis. Megakaryblast matures to mekakarycoyte --\> platelet.
338
When does Fetal hemoglobin predominate
after 8 weeks gestation.
339
Spleen anatomy
Red contains phagocytic cells to make RBC and filter RBCs; white: lymphocytes
340
Risks in splenectomy
sepsis 1) vacciantion before, prophylatic antibiotidies, see doctor if fever above 38.5
341
Saturation of heptoglobin
due to increased hemolysis and leads to oxidation to methemglobin
342
Clonal delection
if IgM on surfae encounters self, it initiates apoptosis. If it doesn't encounter antigen in 24 horus, unlikely to antibody towards self and can surive
343
Where in the body is it acidic?
At tissues - CO2 is produced at tissue due to metabolism, and carbonic anhydrase converts in bloodstream CO2 and water into carbonic acid to eventually convert to bicarbonate and drop the pH
344
Polychromasia
premature release of reticulocytes from marrow - have bluish tint
345
Beta0 thal NBS
F
346
G-6-PD deficiency
X-linked recessive; prevalent in tripical eastern hemisphere, selective resistance to malaria
347
NK cells
recognize basence of normal surface molecules
348
Barts Hb
Y4
349
cortex in llymph node
tightly but motile lymphocytes in follicles
350
Sideroblastic Anemia
imparied production or protoporpyrin or incorporation of iron
351
Platelet Counting range
6-36 fl
352
when does fetus make igG?
3 months old
353
Basophils - immuno
acidic cells, release histamine, Mast cells in tissue; 0.5-1%
354
Acute complications of SCD
Vaso-occlusive pain, acute chest syndrome, infection, spelnic infarction and sequestration, stroke
355
Lab/clinical findings of folate and B12 deficiency
megaloblastic anemia
356
Fecal urobilinogen can go through three pathways
1) fecal mater, 2) kidney for excretion 3) EHC recycling
357
measureable components of CBC?
Hb, Hct, RBC count, MCV, WBC count, Platelet count, mean platelet volume
358
Protein calorie malnutrition laboratory/clinical
variable anemai, normochromic and normocytic
359
Cooperative binding
creates a sigmoidal curve and allows for binding in lungs and release at tissue.
360
Beta Thalassemia lab findings
decrease MCV, increased RBC, MCV/RBC
361
Cathelicidin
bind to negative charged pathogens and bore holes - located on skin/mucosa
362
Bohr effect in Fetal hemoglobin
As blood flows through placenta, H+ is transferred from maternal to fetus circulation to increase pH and and oxygen follows from mother to fetus
363
Cellular response with folate and Vit b12 def
increase cell size, arrest in S phase of mitosis. Cells are degraded and have ineffecitve erythropoiesis.
364
Aplastic crisis
associated with parvovirus B19; causes low retic, severe anemia, pallor; rahs on arms and face
365
Hemoglobin Chesapeak
High affinity Oxygen Hb due to a single point mutation; cauese reduced delivery to tissues and increase EPO release and increased RBC production
366
what amino acids are on outer surface of hb
lysine, arginine, glutamic acid to keep hyrophilic
367
where does heme bind to alpha globin
87th amino acid
368
Rag recombinase
enzyme responsible for looping out random parts of DNA to greate VDJ recombination
369
symptoms for PK deficiency
variable anemia, hemolysis, splenomegaly, gallstone, aplastic crisis
370
Transfusion above 10 g/dl
dangerous cause hyperviscosity
371
Hydrops fetalis
4 deletions, heart dtrain due to decreased blood volumne; subcutaenous edema
372
Treatment of Renal insufficiency
EPO and treatment of co-morbid conditions
373
Beta+ thal NBS
FA
374
Metamyelocyte
must be half diameter, nuclear indentation
375
what causes RBC death?
decreased enzymatic activity, oxidative injury, change in Ca balance, chagnes in membrane carb, antibodies, surface constituents
376
PAMPs
pathagen associated molecular patterns, recognize forein structures through interaction with PRR
377
Too many lymphocytes
lymphocytosis
378
symptoms of G6PD deficiency
acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies
379
Chronic Complications of SCD
Sickle lung disease, pulmonary hypertension, sickle nephropathy; retinopathy, leg ulcers; avascular necrosis
380
lab finding for hereditary spherocytosis
increased retic count, decrased MCV, spherocytes
381
Negative aspects of direct current technology
counts all cells, so includes white cells. Can not discriminate aggregated plateltes, so platelets could be artificially low
382
Where does curve lean towards in gel test for Ab and AgWhere does curve lean towards in gel test for Ab and Ag
Ab (they are larger)
383
lab tests for PK def
mild to severe anemia, increase retic, no specific morphology
384
Hb SB+ Concentrations
25-45 A1; 3% A2; 2-30 HbF; 55-75 HbS
385
What is in urine during increased intravascualr hemolysis
hemosiderin, hemoglobin, methemglobin
386
how to do reticulocyte count?
Flow cytometry
387
Affinity Maturation
with each replication CDR varies, if antibody encounters antigen it stays in the game.
388
lab tests for PK def
mild to severe anemia, increase retic, no specific morphology
389
Epitope
region of antigen that interacts with CDR, discontinous or continous, falt, also called antigenic determinants. Interacts with CDR non-covalenly.
390
C1-esterase inhibitor
regualtes the actiation of C3 so complement pathway is not always activated
391
Cellularity
White spaces - hematopoeitic spaces (100-age). Celularily decreases with age
392
Most severe sickle cell disease
SCD and SB0
393
Hemoglobin amount - SCD with C
10-12 mg/dl
394
Treatmetn of Lead intoxication
chelation of lead
395
Biochemical test for B12 deficiency
Increases erum folate, increasd serum methylmalonic acid, increas serum-2-methyl ctiric acid
396
inheritance of hereditary spherocytosis
75% AD, 25% AR
397
Mast cell fromation
requires SCF or come from the basihilic lineage with IL3
398
PRR
pathogen recognition receptors are on surface or inner membrans (TLR)
399
Classical pathway of complement
antigen binds to igG (2) or IgM (1) to activate allosteric change to FC to allow C1q to bind to activate cascate of 1-4-2-3-5-6-7-8-9
400
Clonal Selection theory
cells make one antibody, choice is randome dependent on outside signals.
401
Immature B cell
cIgM and surface IgM; clonal deletion
402
NK cells
recognize basence of normal surface molecules
403
Dendritic cell mechanism
cytokine,chemokine, stimulate immature DC to phagocytose everything, activate DC enters lymphatic cystem to nearly lymph node, puts digested pathogen on surface as antigen presentingmolecule for T and B cell --\> activates adaptive response.
404
Treatment of Endocrine Disorders
Hormone replacement
405
What contributes to antibody diversity
recombination, exonucleases, terminal deoxynucleotidyl transferase added random NT to N region
406
Isoeletric Focusing
like electrophoryesis; pH gradient imbedded in gel and migrates to isoelectric point
407
blood in lymph node
entry and exit through hilum
408
Splenic Function
clearance of intravascualr particles; adaptive immune resonse: origin of igM
409
IgD (class)
B cell recptor antibody (antibody forming cells)
410
What is % sat at 30 mmHg
60%
411
Renal insufficiency in Anemia
no kidney function means no EPO, so no erythroid proliferation and decrease RBC production
412
lab finding for hereditary spherocytosis
increased retic count, decrased MCV, spherocytes
413
SB0 - NBS
FS
414
Intravascular Hemolysis
breakage of RBC in vascualr system
415
why is myoglobin not a good oxygen carrier
high affinity for oxygen at low oxygen concetnraitons - can't release at tissues.
416
Cold
IgM (some IgG) bind in extremitie and return in warmth to acivate complement and intravascualr hemolysis
417
Maturation of T lymphocyte
T lymphoblast
418
IgD (class)
B cell recptor antibody (antibody forming cells)
419
\>30% methemoglobin
fatigue, lightheaded, headache
420
Partial pressure in lungs
100 mmHg
421
which has neurological features B12 or folate?
B12 - sensory loss, proprioception loss, ataxia, gait distrubances, Babinski reflex, cognitive and emotional changes.
422
Lab finding of AIHA
Increased Retic, increased bilirubin, hemoglobinuria, warm + Dat for igG or Cold + Dat for complement
423
INFbeta
Triggerd by Malignancies and sepsis, to inhibit erythroid proliferation and decrease RBC production
424
Why does transfusion help with beta thalassemia
supresses inate RBC production and marrow expansion
425
igG (classes)
most abundant, passes placent to fetus, longer effect after immunization, half life of three weeks, opsonizing, 2 igG to activate complement
426
Lectin pathway
complement pathway; mannose bidning protein binds to bacterial structures to activate Complement pathway MBP- 4-2-3-5-6-7-8-9
427
Howell-jolly bodies
basophilic nuclear remnants in erthyrocytes - due to damaged or absent spleen
428
what is %sat at 40 mmHg
75%
429
chronic infections that cuase anemia
pulmonary abscesses, meningitis, chronic GU infection
430
how long in storage pool for granulopoiesis
5-7 days beore going into vessles walls in marginated pool or circulation in peripheral blood.
431
Hemoglobin amount - B+ Thal
11-13 mg/dl
432
How does mRNA for antibody form?
splicesome splices out primary RNA to make VDJ u or VDJdelta
433
Too many monocytes
monocytosis
434
Cholelithiasis
gallstones due to increased bilirubin, --\> obstruction
435
lymph flow in lymph node
entry in periphery, through subscapular sinus through middle of node and out of hilum
436
which deficiency is faster, folate or B12?
Weeks to months for folate, very slowly for B12 (years)
437
Acute Chest Syndrome
Rib/vertebral infarction, infection, bone marrow embolysn lead to decreased blood O2, chest pain, fever, decreased hemoglobin
438
IgG Serum
1000 mg/dl; lightest at 150,000 D
439
is serum creatinine a good indiciator of kidney function?
no - can have widely different GFR
440
mature B cell
sIgM and sIgG with same specificity
441
Treatment of Structural Hb Variants
folic acid, splenectomy not curative, no blood transfusion
442
Endocrine disorders that cuase anemia
thyroid, adrenal insufficiency
443
Peyer Patch
lymph node I n gut that underlies mucosa of SI; located by goblet cells. Phacogytose proteins and particles for luminal transport. Determine if food or threat
444
clinical features of Lead intoxification
personality changess, irritability, headache, weakness, ab pain, vomiting
445
Underproduction or normla Hb in thalassemia
Small RBC, low hb concentration, uniform RBC, Increased RBC production
446
Chelation therapy
deferasirox, deferoxamine, deferiprone
447
Lymphopoiesis
vast majority in Bone Marrow; T cells mature in thymus. Lymphoblast --\> lymphocyte
448
Where in the body is it basic?
CO2 is exhaled in the lungs and leads to increased oxygen affinicity
449
Pre-B cell
cytoplasmic IgM (light and eavy)
450
Basophils in Differential
neuclus is obscered by coards, purple, black granules
451
MCHC
Mean Corpsular Hemoglobin Concentration; avg conc of hg in given volume of red cells
452
blood in lymph node
entry and exit through hilum
453
cortex in llymph node
tightly but motile lymphocytes in follicles
454
HbD
punjab; Beta 121 Glu --\> Gln (polar); india, packistan, iran
455
TLR
bind to initiate inflammatioon cascade and ultimately activate NFkB
456
how long do nutrophils spend in periphery
less than 10 years, where they are taken out in urine or feces or by monocytes/macrophages
457
caues of folate deficiency
dietary insufficiency, malabsorption, drugs and toxins, errors in folate metabolism, increased demand, increased metabolism/loss
458
Bohr Effect
Oxygen is held tightly in basic conditions and released in acidic
459
Gamma vs Beta Globin
Differs by 39 amino acids; binds to 2-3 BPG poorly, so it is stabalied in R state and shifts curve to left
460
supportive care, avoid oxidant drugs/food, folate, transfusion if severe
antibodies that cause hemolysis intravascualr and extravascualr
461
30-50% methemoglobin
moderate depression of CNS, achycardia, tachypnea, shortness of breath
462
here does heme bind to beta globin
92nd amino acid
463
Treatment of B12 deficiency
1mg injections weekly/monthly or 2ug orally 2x day
464
Cooley's Clinical manifestations
Bone marrow expansion - hair on end, spelenmegaly, osteopenia, iron overlad
465
igG (classes)
most abundant, passes placent to fetus, longer effect after immunization, half life of three weeks, opsonizing, 2 igG to activate complement
466
methyltetrahydrofolate
is transferred to liver for sotrage, tissue, and undergoes EHC recycling with jejunum
467
Spleen anatomy
Red contains phagocytic cells to make RBC and filter RBCs; white: lymphocytes
468
Risks in splenectomy
sepsis 1) vacciantion before, prophylatic antibiotidies, see doctor if fever above 38.5
469
Sickle cell trait Concentrations
55-60 A1;3% H2;
470
Normocytic anemia types
anemia of chronic disease, sickle cell
471
Polymorphonuclear cells
eosinophils, basophils, neutrophils
472
Allotypic exclusion
like X inactivation of antibodies, recombination can be done if initial antibody is unsuccessful
473
Class switching
V domain stays the same, but splicing occurs at DNA level to cut out other constant regions. Cannot go back to the ones cut out
474
Anemia of Chronic Disease
Chronic infection, chronic inflammation, malignant disease, renal insufficiency, lead intoxification, endocrine disorders
475
Too few lymphocytes
lymphopenia
476
Pre-B cell
cytoplasmic IgM (light and eavy)
477
Lab findings of Chronic infection, inflammation, malignancy
mild/moderate anemia, normochromic/microcytic, dec serum Fe, dec TIBC, normal to increase ferriting, decrease EPO and dec Retic count
478
Retic count - SCD with B0 Thal
5-30%
479
Domains in antibodys
made of 7 Beta sheets for rigidity. The region sin between the sheets form the variable regions. Held together by disulfide bonds
480
laboratory features of renal insufficiency
mod/severe anemia, normochromic, normocytic, EPO dificiency
481
Neutrophils - immuno
fasts moving and move prominetn cell sin adults 40-60%
482
Perinuclear hof
golgi in myelocyte
483
IgE (class)
binds to mast cells and eosinophils, triggers histamine release for allergic reactions; parasite killer
484
Retic count - SCD
5-30%
485
Pathophysiology of SCD
hemolysis, increased endothelial-1, proliferation of smooth muscle, increased adhesion, increased platelet activation
486
Neutrophils - immuno
fasts moving and move prominetn cell sin adults 40-60%
487
MCH calculation
Hb/RBC count
488
how type of iron binds to Hb?
Ferrous 2+
489
Methemoglobin
Ferric form binds to Hb instead of Ferrous. Capacity to carry oxygen is reduced and curve shifts LEFT?
490
Classical pathway of complement
antigen binds to igG (2) or IgM (1) to activate allosteric change to FC to allow C1q to bind to activate cascate of 1-4-2-3-5-6-7-8-9
491
Newborn screening SS
FS
492
Mast cells
Found in tissues rather than peripheral blood, finer granules with one prominent nuclues.
493
Transfusions
dilute out sickle RBC and provides healthy RBCs
494
acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies
supportive care, avoid oxidant drugs/food, folate, transfusion if severe
495
where do dendritic cells gather in lymph node
between cortex and paracrotex
496
where do dendritic cells gather in lymph node
between cortex and paracrotex
497
Alternative complement pathway
cell wall structures in microbes activate C3 directly in absense of antibody (innate)
498
Papin
reduces disulfide bonds to create 2 Fab and 1 Fc
499
Moya Moya fromation in SCD
abnoraml cerebral blood vessesl due to vasculopathy
500
IL-1
stimualted by chronic infection and inflammation to decrease iron and decrase erythropoietin
