Exam 1 Flashcards
Positive cooperativity
oxgen binding to hb alters confirmation to increase affinity for other oxygen molecules
Acute chest syndrome associated complications
kidney and liver failure
How does temp influence saturation curve?
shifts to the right
Vitamin B12 absorption
in stomach with Intrinsic Factors, gets absorbed in ileum and is released from IF
Thrombopoetin
differentiates and matures patelets into periphery, originates from other cells and small component of kidney
Treatment of Chronic infection/inflammation/malignancy
treatment of underlying disease to decrease cytokines and interluekins
Redicualte in circulation
1-2 days
Idiotype
difference in CDR due to specificity.
Pro-Bcell
just heavy chain with VDJu
Calculated Hematocrt
RBC * MCV
Alpha globin in DNA
on CH16, 2 paternal and 2 maternal; Zeta during development
post-capillary venules in lymph node
high-culoidal to react to lymphocytes
too few monocytes
monocytopenia
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
IgD
long hinge region, 5 mg/dl; 180,000 D
Hb Portald 1
Z2Y2
RDW calculation
standard deviaiton of values of MCV
TLR
bind to initiate inflammatioon cascade and ultimately activate NFkB
Allotypic exclusion
like X inactivation of antibodies, recombination can be done if initial antibody is unsuccessful
Hemolytic anemais
Hereditary spherocytosis, enzyme disorders, autoimmune hemolytic anemia
Fe Deficient - Concentrations
99% a1; 2% A2;
MCV
Mean Corpsular Volume, average volume of RBC
Spectrin, ankryn, band 3 alll..
lead to destabalization of lipid bilayer and microspherocytes –> deformability
How does temperature affect oxygen affinity
Higher temperature unloads oxygen easier
Retic count in hemolysis
increases
Immunogen
antigen that generates immune response
symptoms of G6PD deficiency
acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies
Direct Antiglobin testing
IgG, C3d, C4dl tests directly in patient blood
How does dropping the pH shift the Saturation curve
to the right, -lower the affinity of oxygen
monocytes
immature macorphages or dendritic cells comprise 2-8% of WBC
Hb Poole
mutation in gamma chain, phycial unstable variant; infants have hemolytic anema, but it resolves with time.
Characteristics of monopoesis
delicate chormatin pattern, gains vaculoles during maturation, abundance of cyto
Maturation of B lymphoblast
to B lymphocyte to Plasma cell with lots of golig and ER
what factors influence oxgygen dissocation?
pH, temperature, 2,3-BPG
Papin
reduces disulfide bonds to create 2 Fab and 1 Fc
Gestational Hemoglobins
Gower ! And II, and Portland I and II
IgE
extra long C4, 0.2 mg/dl; 190,000 D
HbE
Beta 26 Glu –> Lys; SE asia
Direct Current Technology
blood passes through electrodes and creates resistance in proprotional to cell size. RBC and platlets could be measured simultaneously.
Hypocellular marrow is called_
autoimmmune attack, viral attack
What causes hypercellular marrow?
hypoxia, increase in HGFs
Taut
T hb in low oxygen situations. Four unoccupied sites, binding affinity is low.
Where does megakaryopoiesis take place?
in bone marrow along vascular sinuses and integrates into lumen of vessels and release plasma into bloodstream
post-capillary venules in lymph node
high-culoidal to react to lymphocytes
Germinal center
sites of intense lymphocyte division (b cell). Increase cytoplasm compared to nucleus
Fc
botton region of heavy chains with carbohydrate to prevent collapse
VCAM1
increased in SCD, increased cell adhesion to endoethelium
Maturation of B lymphocyte
to B lymphoblast to Plasma cell with lots of golig and ER
Where does Hb F remain high?
Infants with mothers who have diabetes, hemolytic anemia, bone marrow diseases
Alpha Thal concentations
97 A1; 3% Ab2;
mature B cell
sIgM and sIgG with same specificity
Too many eosinophils
eosinophilia
Monopoesis
Requires M-CSF; monoblast –> promonocyte –> monocyte
Trends in Eosinophils
always 0-6%
lifespan of sickled cell
SC - NBS
FSC
Cholelithiasis
gallstones due to increased bilirubin, –> obstruction
Retic count - SCD with B+ Thal
3-5%
Low Affinity Hb variants Symptoms
mild anemia, cyanosis
What prevents infection in SCD?
penicillin porphylaxis
alpha -thal NBS
Fbarts
primary RNA transcript in antibody genes
made through Cu and Cdelta
Decreased RBC survival in thalassemia
increased retic count, intreacsed intracellular RBC contnets, splenomegaly, bilirubin gallstones, anemia
MCHC calculation
hb/hct
How does mRNA for antibody form?
splicesome splices out primary RNA to make VDJ u or VDJdelta
monocytes
immature macorphages or dendritic cells comprise 2-8% of WBC
NK cells
differentiated B cell, with immunity and survelliance
TNF
stimulated by malignancies and sepsis, to decrease iron and therefore erythropoeitin and inhibit RBC production
what converst bilirubin to glucuronic acid
CYP450 in liver
Splenic Function
clearance of intravascualr particles; adaptive immune resonse: origin of igM
what joins first in VDJ sequence
DJ
Beta Globin in DNA
CH11, Epsilon during development, one copy from each parent
Too many neutrophils
neutrophilia
paracortex in lyph node
not as dense as cortex, houses the T cells
C5A
chemotactic agent to attract bacteria in complement pathway
reticulcoytes time in marrow
3-4 days
MCH
mean corpsular hemoglobin; weight of Hb of the average red cell
what joins first in VDJ sequence
DJ
clinical features of renal insufficiency
fatigue, pallor decreased exercise tolerance, dyspena, tachypena, anemia - unrepalted to kidney dysfunction
Opsonizing
opsonizationÿis a means of identifying the invading particle to the phagocyte; labled with igG and C3B to act as handles for phagocytosis
Infections with SCD is due to
due to spenid dysfunction and aplastic crisis
IgD
long hinge region, 5 mg/dl; 180,000 D
order these in order of sicking potenial - SS, SF, SC, SF
SF
Splenic Sequestration
blood flow into sinusoids is occluded and unable to flow out. Splenomegaly
Complications with hereditary spherocytosis
aplastic crisis and cholelithiasis
IgA
dimer with J chain and secretory component, 400,000D
200 mg/dl
IgG Serum
1000 mg/dl; lightest at 150,000 D
Granulopoiesis
creation of eosinophil, basophils, and neutrophils
calculated components of a CBC
Hct, MCV, mean capsular hb, mean capsular hb conc, RDW, absolute leukocytes
Mononulcear cells make
monocytes and lymphocytes
2,3 - BPG
byrpoduct of anaerobic glycolysys. During increased O2 use, hypoxia, chronic anemia, more is produced because glycolysis increases
what factors increase sickling?
deoxygenation, potassium, dehydration, Hb concentration
DAMPS
damage associated molecular patterns - sense internal damage of our own cells with TLR and attarct phagocytic cells
Complement pathway
completes antibody process by activating C3 to activates membrane attach complex
Immune complex
froms when antibodies bond to antigens and cross link due to divalnce at equal concetnraiton of antibody to antigen.
T Cell and B cell distribution in spleen
T cell surround arterioles and B cells surround them
Endothelial-1
vasoconstrictor, increases in SCD
Microcytic anemia types
Fe deficiency, thalassemia, lead poisoning
when does fetus make igG?
3 months old
Direct Antiglobin testing
IgG, C3d, C4dl tests directly in patient blood
how is a differential performed?
blod film (wdge, cover glass, spinner), air dry and fix with MeOH, wright-giemsa stain
Lab finding of AIHA
Increased Retic, increased bilirubin, hemoglobinuria, warm + Dat for igG or Cold + Dat for complement
paracortex in lyph node
not as dense as cortex, houses the T cells
Hb Chesapeak symptoms
hypoxia, generally well affected and don’t need treatment
Hb Zurich
point muations to increase binding to CO
Eosinophils in Differential
Bi-lobed nucleus, large red granuals
igM
pentamer, extra long C4, S-S bonds and One J chain, 900,000D; 100 mgdl
seg
muliple lobes with chormatin band, small size, pink cyto
Too many basophils
basophilia
Absorption of Folate
in jejunum where it is hydrolyzed, reduced, methylated into methyltetrahydrofolate.
what is practicla reference interval in CBC?
mean +- 2 SD
IgM (classes)
immunization to activatio of complement, V=10, but binds to 2. vicsous, only ab made in fetus
Immunological Aging
as we age, our memory cells outnumber our na‹ve cells and cant respond to new antigens.
T Cell and B cell distribution in spleen
T cell surround arterioles and B cells surround them
Opsonizing
opsonizationÿis a means of identifying the invading particle to the phagocyte; labled with igG and C3B to act as handles for phagocytosis
Clonal Selection theory
cells make one antibody, choice is randome dependent on outside signals.
HBO
Arab; Beta 121 Glu –> lys (basic); Africa, Middle east
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
Lymphocytes in differential
scant cyto with dense, round nuclus
Cyanosis
blue/grey color skin and mucus membrane; Too must deoxyhemoglobin 1.5 g/dl methemoglobin (8-12%); sulhemoglobin >0.5 g/dl
Hb at birth
65-95% Hb F and 20% HbA
Warm
IgG, poor complement activity activates spenic macrophages and extravacualr hemolysis
when does fetus make igM?
3 months gestation
Basophils - immuno
acidic cells, release histamine, Mast cells in tissue; 0.5-1%
Fetal Hemoglobin
alpha2gamma2
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.
Leukocyte absolute count
Diff% * WBC / 100
HbS
Beta 6 Glu –> Val (acid to hydrophobic); africa, india, med
Endocrine disorders lab findings
mormochronic and cytic, decreased retic count
AS - NBS
FAS
MCV calculation
Hct/RBC count
Humoral
antibody mediated immunity
What contributes to antibody diversity
recombination, exonucleases, terminal deoxynucleotidyl transferase added random NT to N region
Protein Calorie Malnutrition
anemia due to lack of protein and calories
what is %sat at 60 mmHg
90%
Normocytic anemia types
anemia of chronic disease, sickle cell
Symptoms of AIHA
acute or chronic anemia, pallor, jaundice, dark urine, splenomegaly
Splenic InfarctioN
insufficient bloodflow to spleen (usually by age 4 in SCD)
Iron Deficienty lab finidngs
decreased MCV, RBC, MCV/RBC >13, incrase RDW, Decreased ferritin, normal electrophorysis, response to Fe
Immune complex
froms when antibodies bond to antigens and cross link due to divalnce at equal concetnraiton of antibody to antigen.
EB+
FEA
G-CSF
helps early blast stage differenitiating into myeloid lineage as opposed to lmphoid.
C5A
chemotactic agent to attract bacteria in complement pathway
Hematocrit
erythrocote volume/whole blood volume - centrifugation
pathway for malignancis/sepsis in anemia
production of TNF and Interfereon Beta
what does manual differential add?
morphology of RBC, WBC, platelets, abnormalities in peripheral blood, relative or absolute quantification of WBC populations
Hemopexin
binds to free heme from methemogobin to be processed in liver to make bilirubin
Fab
top part of one heavy and one light chain
Methemoglobin - how do you get it?
acquired or genetic
what kind of infection is caused by SCD
encapsulated bacteremia - strep, hemophilus influenza, meningococcus
Affinity Maturation
with each replication CDR varies, if antibody encounters antigen it stays in the game.
igM
pentamer, extra long C4, S-S bonds and One J chain, 900,000D; 100 mgdl
Hemoglobin amount - SCD trait
14-16 mg/dl
Maturation of T lymphocyte
T lymphoblast
How long does MVC take to get back to normal in B12/Folate defidiency
months
How long does Hb take to get back to normal in B12/Folate defidiency
1-2 weeks
when does fetus make igM?
3 months gestation
Sickel Mephropathy
10-15%; hyperfiltration and enlarged glomeruli, porteinuria
what can you measure with flow cytometry?
Reticulocyte count, nucleated RBC types, platelet count
Treatment of hereditary spherocytosis
supportive care, folate, splenectomy
Epitope
region of antigen that interacts with CDR, discontinous or continous, falt, also called antigenic determinants. Interacts with CDR non-covalenly.
what globin chain makes antibodies
gamma (called immunoglobulin
Rag recombinase
enzyme responsible for looping out random parts of DNA to greate VDJ recombination
Symptoms of AIHA
acute or chronic anemia, pallor, jaundice, dark urine, splenomegaly
medulla role lymph node
filter
measuring MCV
mean height of the voltage pulse during RBC count
hemoglobincyanide method
combine bood with potassium cyanide and potassium ferricyanide, product absorbs light at 540 nm. Can detect how much Hb based on absorbance.
macrocytic anemia types
vit B12 and volate deficience
HbH
B4
Retic count - HbSC
3-5%
HbEE
FE
Tolerogen
antigen that does not ellicent and immune response
Less severe sickle cell types
SB+, SC, trait (no anemia)
how to limit iron loading
erythroectapheresis and phlebotomy prior to transfusion
Mitotic pool of granulopoiesis
4-5 cell divisions, where they enter the storage pool with no more cell division
what globin chain makes antibodies
gamma (called immunoglobulin
Retic count - SC Trait
1-2%
Pulmonary hypertension
increased pressure on pulmonary arteries, most often due to sickle lung disease
What type of bilirubin is expelled in feces?
fecal urobilinogen
Lab findings of lead intox
mild/mod anemia, microcytosis, mild hypochormia, basophilic stippling, increase zinc protoporphyrin.
Sickle lung disease
25-40% of SCD; preogressive obliteration of pulmonary vasculature; leading cuase of death in adults with SCD
Immunological Aging
as we age, our memory cells outnumber our na‹ve cells and cant respond to new antigens.
Alternative complement pathway
cell wall structures in microbes activate C3 directly in absense of antibody (innate)
HbC
Beta 6 Glu –> Lys (acid to base); west africa
RDW
Red blood cell distribution width
order of antibody sequence for DNA
VDJ
Hb A2
alpha 2 delta 2
iron in RBC transfusion
1ml has 1 mg Fe; 3-4 g Fe/yr
Idiotype
difference in CDR due to specificity.
Bilirubin in extravascualr hemolysis
porphyrin ring forms bilirubin, which is conjugated to glucoronic acid in liver and then in bowel is converted to urobilinogen
Complement pathway
completes antibody process by activating C3 to activates membrane attach complex
Pathophysiology of thalassemia
Imbalance alpha and beta, excess globin participate, ineffecient erythropoesis
Monocytes in differential
largets, irregulat nulcues, fine granuals, vacuolated cytoplasm
Complications with hereditary spherocytosis
aplastic crisis and cholelithiasis
Eosinophils - immuno
basic cells, bind to major basic proein, kill parasites. 1-4%
Sickle Dex
hemolyze blood with reducing agent, and release Hb. Sickle is less soluble. Can detect SBD, but not type of disease
Hb Portland 2
Z2 B2
half life of igG?
3 weeks
What is P50 or hb 50% saturated?
27 mmHg
Hydroxyurea and bytyrate
fetal Hb induction; reduces sickle hb polymerization, decreases WBC count