Exam 1 Flashcards

1
Q

Positive cooperativity

A

oxgen binding to hb alters confirmation to increase affinity for other oxygen molecules

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2
Q

Acute chest syndrome associated complications

A

kidney and liver failure

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3
Q

How does temp influence saturation curve?

A

shifts to the right

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4
Q

Vitamin B12 absorption

A

in stomach with Intrinsic Factors, gets absorbed in ileum and is released from IF

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5
Q

Thrombopoetin

A

differentiates and matures patelets into periphery, originates from other cells and small component of kidney

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6
Q

Treatment of Chronic infection/inflammation/malignancy

A

treatment of underlying disease to decrease cytokines and interluekins

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7
Q

Redicualte in circulation

A

1-2 days

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8
Q

Idiotype

A

difference in CDR due to specificity.

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9
Q

Pro-Bcell

A

just heavy chain with VDJu

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10
Q

Calculated Hematocrt

A

RBC * MCV

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11
Q

Alpha globin in DNA

A

on CH16, 2 paternal and 2 maternal; Zeta during development

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12
Q

post-capillary venules in lymph node

A

high-culoidal to react to lymphocytes

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13
Q

too few monocytes

A

monocytopenia

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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

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15
Q

IgD

A

long hinge region, 5 mg/dl; 180,000 D

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16
Q

Hb Portald 1

A

Z2Y2

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17
Q

RDW calculation

A

standard deviaiton of values of MCV

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18
Q

TLR

A

bind to initiate inflammatioon cascade and ultimately activate NFkB

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19
Q

Allotypic exclusion

A

like X inactivation of antibodies, recombination can be done if initial antibody is unsuccessful

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20
Q

Hemolytic anemais

A

Hereditary spherocytosis, enzyme disorders, autoimmune hemolytic anemia

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21
Q

Fe Deficient - Concentrations

A

99% a1; 2% A2;

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22
Q

MCV

A

Mean Corpsular Volume, average volume of RBC

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23
Q

Spectrin, ankryn, band 3 alll..

A

lead to destabalization of lipid bilayer and microspherocytes –> deformability

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24
Q

How does temperature affect oxygen affinity

A

Higher temperature unloads oxygen easier

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25
Q

Retic count in hemolysis

A

increases

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26
Q

Immunogen

A

antigen that generates immune response

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27
Q

symptoms of G6PD deficiency

A

acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies

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28
Q

Direct Antiglobin testing

A

IgG, C3d, C4dl tests directly in patient blood

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29
Q

How does dropping the pH shift the Saturation curve

A

to the right, -lower the affinity of oxygen

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30
Q

monocytes

A

immature macorphages or dendritic cells comprise 2-8% of WBC

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31
Q

Hb Poole

A

mutation in gamma chain, phycial unstable variant; infants have hemolytic anema, but it resolves with time.

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32
Q

Characteristics of monopoesis

A

delicate chormatin pattern, gains vaculoles during maturation, abundance of cyto

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33
Q

Maturation of B lymphoblast

A

to B lymphocyte to Plasma cell with lots of golig and ER

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34
Q

what factors influence oxgygen dissocation?

A

pH, temperature, 2,3-BPG

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35
Q

Papin

A

reduces disulfide bonds to create 2 Fab and 1 Fc

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36
Q

Gestational Hemoglobins

A

Gower ! And II, and Portland I and II

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37
Q

IgE

A

extra long C4, 0.2 mg/dl; 190,000 D

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38
Q

HbE

A

Beta 26 Glu –> Lys; SE asia

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39
Q

Direct Current Technology

A

blood passes through electrodes and creates resistance in proprotional to cell size. RBC and platlets could be measured simultaneously.

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40
Q

Hypocellular marrow is called_

A

autoimmmune attack, viral attack

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41
Q

What causes hypercellular marrow?

A

hypoxia, increase in HGFs

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42
Q

Taut

A

T hb in low oxygen situations. Four unoccupied sites, binding affinity is low.

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43
Q

Where does megakaryopoiesis take place?

A

in bone marrow along vascular sinuses and integrates into lumen of vessels and release plasma into bloodstream

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44
Q

post-capillary venules in lymph node

A

high-culoidal to react to lymphocytes

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45
Q

Germinal center

A

sites of intense lymphocyte division (b cell). Increase cytoplasm compared to nucleus

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46
Q

Fc

A

botton region of heavy chains with carbohydrate to prevent collapse

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47
Q

VCAM1

A

increased in SCD, increased cell adhesion to endoethelium

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48
Q

Maturation of B lymphocyte

A

to B lymphoblast to Plasma cell with lots of golig and ER

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49
Q

Where does Hb F remain high?

A

Infants with mothers who have diabetes, hemolytic anemia, bone marrow diseases

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50
Q

Alpha Thal concentations

A

97 A1; 3% Ab2;

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51
Q

mature B cell

A

sIgM and sIgG with same specificity

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52
Q

Too many eosinophils

A

eosinophilia

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53
Q

Monopoesis

A

Requires M-CSF; monoblast –> promonocyte –> monocyte

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54
Q

Trends in Eosinophils

A

always 0-6%

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55
Q

lifespan of sickled cell

A
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56
Q

SC - NBS

A

FSC

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57
Q

Cholelithiasis

A

gallstones due to increased bilirubin, –> obstruction

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58
Q

Retic count - SCD with B+ Thal

A

3-5%

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59
Q

Low Affinity Hb variants Symptoms

A

mild anemia, cyanosis

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60
Q

What prevents infection in SCD?

A

penicillin porphylaxis

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61
Q

alpha -thal NBS

A

Fbarts

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62
Q

primary RNA transcript in antibody genes

A

made through Cu and Cdelta

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63
Q

Decreased RBC survival in thalassemia

A

increased retic count, intreacsed intracellular RBC contnets, splenomegaly, bilirubin gallstones, anemia

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64
Q

MCHC calculation

A

hb/hct

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65
Q

How does mRNA for antibody form?

A

splicesome splices out primary RNA to make VDJ u or VDJdelta

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66
Q

monocytes

A

immature macorphages or dendritic cells comprise 2-8% of WBC

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67
Q

NK cells

A

differentiated B cell, with immunity and survelliance

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68
Q

TNF

A

stimulated by malignancies and sepsis, to decrease iron and therefore erythropoeitin and inhibit RBC production

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69
Q

what converst bilirubin to glucuronic acid

A

CYP450 in liver

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70
Q

Splenic Function

A

clearance of intravascualr particles; adaptive immune resonse: origin of igM

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71
Q

what joins first in VDJ sequence

A

DJ

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72
Q

Beta Globin in DNA

A

CH11, Epsilon during development, one copy from each parent

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73
Q

Too many neutrophils

A

neutrophilia

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74
Q

paracortex in lyph node

A

not as dense as cortex, houses the T cells

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75
Q

C5A

A

chemotactic agent to attract bacteria in complement pathway

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76
Q

reticulcoytes time in marrow

A

3-4 days

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77
Q

MCH

A

mean corpsular hemoglobin; weight of Hb of the average red cell

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78
Q

what joins first in VDJ sequence

A

DJ

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79
Q

clinical features of renal insufficiency

A

fatigue, pallor decreased exercise tolerance, dyspena, tachypena, anemia - unrepalted to kidney dysfunction

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80
Q

Opsonizing

A

opsonizationÿis a means of identifying the invading particle to the phagocyte; labled with igG and C3B to act as handles for phagocytosis

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81
Q

Infections with SCD is due to

A

due to spenid dysfunction and aplastic crisis

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82
Q

IgD

A

long hinge region, 5 mg/dl; 180,000 D

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83
Q

order these in order of sicking potenial - SS, SF, SC, SF

A

SF

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84
Q

Splenic Sequestration

A

blood flow into sinusoids is occluded and unable to flow out. Splenomegaly

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85
Q

Complications with hereditary spherocytosis

A

aplastic crisis and cholelithiasis

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86
Q

IgA

A

dimer with J chain and secretory component, 400,000D

200 mg/dl

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87
Q

IgG Serum

A

1000 mg/dl; lightest at 150,000 D

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88
Q

Granulopoiesis

A

creation of eosinophil, basophils, and neutrophils

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89
Q

calculated components of a CBC

A

Hct, MCV, mean capsular hb, mean capsular hb conc, RDW, absolute leukocytes

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90
Q

Mononulcear cells make

A

monocytes and lymphocytes

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91
Q

2,3 - BPG

A

byrpoduct of anaerobic glycolysys. During increased O2 use, hypoxia, chronic anemia, more is produced because glycolysis increases

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92
Q

what factors increase sickling?

A

deoxygenation, potassium, dehydration, Hb concentration

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93
Q

DAMPS

A

damage associated molecular patterns - sense internal damage of our own cells with TLR and attarct phagocytic cells

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94
Q

Complement pathway

A

completes antibody process by activating C3 to activates membrane attach complex

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95
Q

Immune complex

A

froms when antibodies bond to antigens and cross link due to divalnce at equal concetnraiton of antibody to antigen.

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96
Q

T Cell and B cell distribution in spleen

A

T cell surround arterioles and B cells surround them

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97
Q

Endothelial-1

A

vasoconstrictor, increases in SCD

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98
Q

Microcytic anemia types

A

Fe deficiency, thalassemia, lead poisoning

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99
Q

when does fetus make igG?

A

3 months old

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100
Q

Direct Antiglobin testing

A

IgG, C3d, C4dl tests directly in patient blood

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101
Q

how is a differential performed?

A

blod film (wdge, cover glass, spinner), air dry and fix with MeOH, wright-giemsa stain

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102
Q

Lab finding of AIHA

A

Increased Retic, increased bilirubin, hemoglobinuria, warm + Dat for igG or Cold + Dat for complement

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103
Q

paracortex in lyph node

A

not as dense as cortex, houses the T cells

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104
Q

Hb Chesapeak symptoms

A

hypoxia, generally well affected and don’t need treatment

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105
Q

Hb Zurich

A

point muations to increase binding to CO

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106
Q

Eosinophils in Differential

A

Bi-lobed nucleus, large red granuals

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107
Q

igM

A

pentamer, extra long C4, S-S bonds and One J chain, 900,000D; 100 mgdl

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108
Q

seg

A

muliple lobes with chormatin band, small size, pink cyto

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109
Q

Too many basophils

A

basophilia

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110
Q

Absorption of Folate

A

in jejunum where it is hydrolyzed, reduced, methylated into methyltetrahydrofolate.

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111
Q

what is practicla reference interval in CBC?

A

mean +- 2 SD

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112
Q

IgM (classes)

A

immunization to activatio of complement, V=10, but binds to 2. vicsous, only ab made in fetus

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113
Q

Immunological Aging

A

as we age, our memory cells outnumber our na‹ve cells and cant respond to new antigens.

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114
Q

T Cell and B cell distribution in spleen

A

T cell surround arterioles and B cells surround them

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115
Q

Opsonizing

A

opsonizationÿis a means of identifying the invading particle to the phagocyte; labled with igG and C3B to act as handles for phagocytosis

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116
Q

Clonal Selection theory

A

cells make one antibody, choice is randome dependent on outside signals.

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117
Q

HBO

A

Arab; Beta 121 Glu –> lys (basic); Africa, Middle east

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118
Q

Domains in antibodys

A

made of 7 Beta sheets for rigidity. The region sin between the sheets form the variable regions. Held together by disulfide bonds

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119
Q

Lymphocytes in differential

A

scant cyto with dense, round nuclus

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120
Q

Cyanosis

A

blue/grey color skin and mucus membrane; Too must deoxyhemoglobin 1.5 g/dl methemoglobin (8-12%); sulhemoglobin >0.5 g/dl

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121
Q

Hb at birth

A

65-95% Hb F and 20% HbA

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122
Q

Warm

A

IgG, poor complement activity activates spenic macrophages and extravacualr hemolysis

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123
Q

when does fetus make igM?

A

3 months gestation

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124
Q

Basophils - immuno

A

acidic cells, release histamine, Mast cells in tissue; 0.5-1%

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125
Q

Fetal Hemoglobin

A

alpha2gamma2

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126
Q

How is bone marrow biopsy performed?

A

through ilica crest, suction to pull out reiculyn fibers; use a core to pull out a piece of marrow that contains bone and cells.

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127
Q

Leukocyte absolute count

A

Diff% * WBC / 100

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128
Q

HbS

A

Beta 6 Glu –> Val (acid to hydrophobic); africa, india, med

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129
Q

Endocrine disorders lab findings

A

mormochronic and cytic, decreased retic count

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130
Q

AS - NBS

A

FAS

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131
Q

MCV calculation

A

Hct/RBC count

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132
Q

Humoral

A

antibody mediated immunity

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133
Q

What contributes to antibody diversity

A

recombination, exonucleases, terminal deoxynucleotidyl transferase added random NT to N region

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134
Q

Protein Calorie Malnutrition

A

anemia due to lack of protein and calories

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135
Q

what is %sat at 60 mmHg

A

90%

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136
Q

Normocytic anemia types

A

anemia of chronic disease, sickle cell

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137
Q

Symptoms of AIHA

A

acute or chronic anemia, pallor, jaundice, dark urine, splenomegaly

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138
Q

Splenic InfarctioN

A

insufficient bloodflow to spleen (usually by age 4 in SCD)

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139
Q

Iron Deficienty lab finidngs

A

decreased MCV, RBC, MCV/RBC >13, incrase RDW, Decreased ferritin, normal electrophorysis, response to Fe

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140
Q

Immune complex

A

froms when antibodies bond to antigens and cross link due to divalnce at equal concetnraiton of antibody to antigen.

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141
Q

EB+

A

FEA

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142
Q

G-CSF

A

helps early blast stage differenitiating into myeloid lineage as opposed to lmphoid.

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143
Q

C5A

A

chemotactic agent to attract bacteria in complement pathway

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144
Q

Hematocrit

A

erythrocote volume/whole blood volume - centrifugation

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145
Q

pathway for malignancis/sepsis in anemia

A

production of TNF and Interfereon Beta

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146
Q

what does manual differential add?

A

morphology of RBC, WBC, platelets, abnormalities in peripheral blood, relative or absolute quantification of WBC populations

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147
Q

Hemopexin

A

binds to free heme from methemogobin to be processed in liver to make bilirubin

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148
Q

Fab

A

top part of one heavy and one light chain

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149
Q

Methemoglobin - how do you get it?

A

acquired or genetic

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150
Q

what kind of infection is caused by SCD

A

encapsulated bacteremia - strep, hemophilus influenza, meningococcus

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151
Q

Affinity Maturation

A

with each replication CDR varies, if antibody encounters antigen it stays in the game.

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152
Q

igM

A

pentamer, extra long C4, S-S bonds and One J chain, 900,000D; 100 mgdl

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153
Q

Hemoglobin amount - SCD trait

A

14-16 mg/dl

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154
Q

Maturation of T lymphocyte

A

T lymphoblast

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155
Q

How long does MVC take to get back to normal in B12/Folate defidiency

A

months

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156
Q

How long does Hb take to get back to normal in B12/Folate defidiency

A

1-2 weeks

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157
Q

when does fetus make igM?

A

3 months gestation

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158
Q

Sickel Mephropathy

A

10-15%; hyperfiltration and enlarged glomeruli, porteinuria

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159
Q

what can you measure with flow cytometry?

A

Reticulocyte count, nucleated RBC types, platelet count

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160
Q

Treatment of hereditary spherocytosis

A

supportive care, folate, splenectomy

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161
Q

Epitope

A

region of antigen that interacts with CDR, discontinous or continous, falt, also called antigenic determinants. Interacts with CDR non-covalenly.

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162
Q

what globin chain makes antibodies

A

gamma (called immunoglobulin

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163
Q

Rag recombinase

A

enzyme responsible for looping out random parts of DNA to greate VDJ recombination

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164
Q

Symptoms of AIHA

A

acute or chronic anemia, pallor, jaundice, dark urine, splenomegaly

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165
Q

medulla role lymph node

A

filter

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166
Q

measuring MCV

A

mean height of the voltage pulse during RBC count

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167
Q

hemoglobincyanide method

A

combine bood with potassium cyanide and potassium ferricyanide, product absorbs light at 540 nm. Can detect how much Hb based on absorbance.

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168
Q

macrocytic anemia types

A

vit B12 and volate deficience

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169
Q

HbH

A

B4

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170
Q

Retic count - HbSC

A

3-5%

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171
Q

HbEE

A

FE

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172
Q

Tolerogen

A

antigen that does not ellicent and immune response

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173
Q

Less severe sickle cell types

A

SB+, SC, trait (no anemia)

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174
Q

how to limit iron loading

A

erythroectapheresis and phlebotomy prior to transfusion

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175
Q

Mitotic pool of granulopoiesis

A

4-5 cell divisions, where they enter the storage pool with no more cell division

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176
Q

what globin chain makes antibodies

A

gamma (called immunoglobulin

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177
Q

Retic count - SC Trait

A

1-2%

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178
Q

Pulmonary hypertension

A

increased pressure on pulmonary arteries, most often due to sickle lung disease

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179
Q

What type of bilirubin is expelled in feces?

A

fecal urobilinogen

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180
Q

Lab findings of lead intox

A

mild/mod anemia, microcytosis, mild hypochormia, basophilic stippling, increase zinc protoporphyrin.

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181
Q

Sickle lung disease

A

25-40% of SCD; preogressive obliteration of pulmonary vasculature; leading cuase of death in adults with SCD

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182
Q

Immunological Aging

A

as we age, our memory cells outnumber our na‹ve cells and cant respond to new antigens.

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183
Q

Alternative complement pathway

A

cell wall structures in microbes activate C3 directly in absense of antibody (innate)

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184
Q

HbC

A

Beta 6 Glu –> Lys (acid to base); west africa

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185
Q

RDW

A

Red blood cell distribution width

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186
Q

order of antibody sequence for DNA

A

VDJ

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187
Q

Hb A2

A

alpha 2 delta 2

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188
Q

iron in RBC transfusion

A

1ml has 1 mg Fe; 3-4 g Fe/yr

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189
Q

Idiotype

A

difference in CDR due to specificity.

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190
Q

Bilirubin in extravascualr hemolysis

A

porphyrin ring forms bilirubin, which is conjugated to glucoronic acid in liver and then in bowel is converted to urobilinogen

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191
Q

Complement pathway

A

completes antibody process by activating C3 to activates membrane attach complex

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192
Q

Pathophysiology of thalassemia

A

Imbalance alpha and beta, excess globin participate, ineffecient erythropoesis

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193
Q

Monocytes in differential

A

largets, irregulat nulcues, fine granuals, vacuolated cytoplasm

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194
Q

Complications with hereditary spherocytosis

A

aplastic crisis and cholelithiasis

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195
Q

Eosinophils - immuno

A

basic cells, bind to major basic proein, kill parasites. 1-4%

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196
Q

Sickle Dex

A

hemolyze blood with reducing agent, and release Hb. Sickle is less soluble. Can detect SBD, but not type of disease

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197
Q

Hb Portland 2

A

Z2 B2

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198
Q

half life of igG?

A

3 weeks

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199
Q

What is P50 or hb 50% saturated?

A

27 mmHg

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200
Q

Hydroxyurea and bytyrate

A

fetal Hb induction; reduces sickle hb polymerization, decreases WBC count

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201
Q

Granulopoiesis starts with_

A

myloblast to promeylocytes that splits into the three idfferent lineages as bands and then seg.

202
Q

Cathelicidin

A

bind to negative charged pathogens and bore holes - located on skin/mucosa

203
Q

Allotype

A

differences in antibody due to mom or dad, in non-CDR region

204
Q

Beta thal trait concentrations

A

94 A1; 4% A2; 2% HbF

205
Q

EB0

A

FE

206
Q

Anemia Concentration - normal

A

97% A1 and 3$% A2;

207
Q

SB+ = NBS

A

FSA

208
Q

Microcytic anemia types

A

Fe deficiency, thalassemia, lead poisoning

209
Q

Heptoglobin

A

plasma proin that binds to HB dimer that is created in intravascular hemolysis; removed from circulation in liver

210
Q

order of antibody sequence for DNA

A

VDJ

211
Q

erythroceytapheresis

A

erytrocytes separate from whole blood

212
Q

Neutrphils in Differential

A

Acidic cytoplasm, 2-5 distinct lobular nuclues

213
Q

Lymphocytes - immuno

A

T and b cells; prominent WBC in children; 20-40%

214
Q

Vaso-occlusive Pain

A

acute complication of SCD; pain in muslces and back and require opiates and antiinflammatory.

215
Q

when are transfusions used?

A

only when severity has potential for cardiovascualr decompensation

216
Q

half life of igG?

A

3 weeks

217
Q

50-60% methemoglobin

A

stupor, bradycardia, respiratory depression, convulsions, acidosis

218
Q

is normal good?

A

in certain diseases tests are expected to be abnormal. Failure to chagne is bodys inability to respond approprioately.

219
Q

Hemoglobin amount - SCD

A

6-9 mg/dl

220
Q

Causes of V12 deficiency

A

pernicicious anemia - autoimmune

221
Q

Biochemical test of Folate Deficiency

A

decreased serum folate.

222
Q

RBC range for Direct Current

A

35-250 fl

223
Q

Schlling Test

A

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
Q

lab finding for hereditary spherocytosis

A

increased retic count, decrased MCV, spherocytes

225
Q

Where does curve lean towards in gel test for Ab and AgWhere does curve lean towards in gel test for Ab and Ag

A

Ab (they are larger)

226
Q

What malignant diseases cause anemia

A

carcinoma, hodgkins disease

227
Q

what causes increase iron absorption

A

ineffecte RBC production, low hepcidin, mutations in HFE

228
Q

stroke in SCD

A

cellular adhesion, loss of vasoregulation by increased endothelial 1 release, and decreased NO.

229
Q

SCD concentrations

A

2-30% HbF 70-98% HbS

230
Q

Sickle Cell Trait Risks

A

kidney complications: microscopic hematuria, renal papillary necrosis, isosthenia, chronic kidney disease; blood clot risk; splenic infarction, rhadomyolysis

231
Q

Cold

A

IgM (some IgG) bind in extremitie and return in warmth to acivate complement and intravascualr hemolysis

232
Q

Polymorphonuclear cells

A

eosinophils, basophils, neutrophils

233
Q

use of myoglobin

A

oxygen storage in muscle

234
Q

Alpha thalassemia lab findings

A

decrease MCV, increased RBC, MCV/RBC

235
Q

Class switching

A

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
Q

HbH thalassmeia

A

3 deletions; moderate anemia, makes B4 to compensate, transfusion depent on stress

237
Q

Methemoglobin symptoms

A

asymptomatic, but low sat; dark blood when mixed with air.

238
Q

How does increasing pH shift the saturation curve?

A

Shift to the left - increased affinity

239
Q

Hydroxyurea and bytyrate

A

inductio of HbF

240
Q

primary RNA transcript in antibody genes

A

made through Cu and Cdelta

241
Q

PAMPs

A

pathagen associated molecular patterns, recognize forein structures through interaction with PRR

242
Q

IgA

A

dimer with J chain and secretory component, 400,000D

243
Q

HB SB0 concentrations

A

3% A2; HbF 2-30; S 70-98

244
Q

macrocytic anemia types

A

vit B12 and volate deficience

245
Q

PRR

A

pathogen recognition receptors are on surface or inner membrans (TLR)

246
Q

symptoms for PK deficiency

A

variable anemia, hemolysis, splenomegaly, gallstone, aplastic crisis

247
Q

DAMPS

A

damage associated molecular patterns - sense internal damage of our own cells with TLR and attarct phagocytic cells

248
Q

Beta thal and Fe deficient concentrations

A

96 A1; 3% A2; 1% HbF

249
Q

gallstones due to increased bilirubin, –> obstruction

A

decreases pyruvate formation and decreases ATP production; loss of membrane plascticity and increased rigidity.

250
Q

Humoral

A

antibody mediated immunity

251
Q

Types of anemia in Hereditary Spherocytosis

A

5% sever; 60-75% moderate; 20% mild

252
Q

methemalbumin in hemolytic anemia

A

increases

253
Q

Lectin pathway

A

complement pathway; mannose bidning protein binds to bacterial structures to activate Complement pathway MBP- 4-2-3-5-6-7-8-9

254
Q

Sideroblastic anemia clinical/lab

A

variable anemia, hypochromia, microcytosis, accumulation of iron in mito

255
Q

G-6PD Def mechanism

A

deecrased production of NADH, reduction in glutathione, and protecton from hydrogen peroxide.

256
Q

medulla role lymph node

A

filter

257
Q

clinical features of Chornic infection, inflammation, malignancy

A

dependent on underlying disease

258
Q

Current mothod of measuring Hb

A

lyse RBC and WBC, alter globin and oxidize heme, sodium lauryl sulphate binds to form colored complex and measure the absorbance.

259
Q

how does chronic inflammation/infection cause anemia?

A

stimulates IL1 and interferon gamma

260
Q

Neutrophil killing

A

1) phagocytosis or 2) degranulation (spews out granules into enironment to cause necrosis and inflammation)

261
Q

Gower 2

A

alpha2E2

262
Q

Warm

A

IgG, poor complement activity activates spenic macrophages and extravacualr hemolysis

263
Q

Risks in transfusion

A

infection, allo-immunization; iron overload

264
Q

Immunogen

A

antigen that generates immune response

265
Q

Iron loss per day

A

1mg/day; fixed; sloughed mucosa, meses

266
Q

iron overload toxicity

A

ears, hearing, bone, liver, kidney

267
Q

Trend of neutrophils over lifetime?

A

45-50% at birth until 2-12 years old increase to 40-75%

268
Q

Treatmetn of acute chest syndrome

A

transfusion to maintain 9-10 mg/dl, induction of HbF, bone marrow transplant

269
Q

what happens in thalassemia

A

Anemai, bone marrow expansion, extramedullary hematopoesis

270
Q

how does pH affect O2 dissociation?

A

Oxygen is held tightly in basic conditions and released in acidic

271
Q

Immature B cell

A

cIgM and surface IgM; clonal deletion

272
Q

heptoserum levels in hemolysis anemia

A

decrases

273
Q

Hereditary Spherocytosis

A

most often due to spectrin deficiency, causes anemia jaundice, splenomegaly. Microspherocyte and osmotic fragility

274
Q

Monopoesis circulation

A

marginal pool for 20 days and can go into tissue to become macrophages; circulation is short lived.

275
Q

Mononulcear cells make

A

monocytes and lymphocytes

276
Q

Retinopathy

A

causes neovascularization (sea fan formation) and venular occlusion (sea fan infarction); redintal detachment, hemorrahge, and blindness

277
Q

Too few neutrophils

A

neutropenia

278
Q

How long does blood count take to get back to normal in B12/Folate defidiency

A

8 weeks

279
Q

Hemoglobin amount - Sickle B0 Thal

A

6-9 mg/dl

280
Q

Fc

A

botton region of heavy chains with carbohydrate to prevent collapse

281
Q

Brend of Bands over lifetime?

A

always 0-9%

282
Q

Allotype

A

differences in antibody due to mom or dad, in non-CDR region

283
Q

Transcoalbumin BP II

A

binds to Vitamin B12 after ileal abosprtion and promotes liver sotrage or transfer to tissue (bone marrow).

284
Q

oxidation of Hb_ from G6PD deficience

A

denatures Hb so it attaches to membrane to create heinz bodies, trapping/deformability and extravascualr hemolysis

285
Q

Clinical manifestations of thalassmeia

A

Underproduction of normal Hb and decreaesd RBC survival

286
Q

Eosinophils - immuno

A

basic cells, bind to major basic proein, kill parasites. 1-4%

287
Q

Lead intoxification and anemia

A

lead inhbits protoporphyrin ring syntehsis and iron from forming hemoglobin

288
Q

G-6-PD deficiency

A

X-linked recessive; prevalent in tripical eastern hemisphere, selective resistance to malaria

289
Q

Peyer Patch

A

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
Q

IgE

A

extra long C4, 0.2 mg/dl; 190,000 D

291
Q

IgE (class)

A

binds to mast cells and eosinophils, triggers histamine release for allergic reactions; parasite killer

292
Q

hematologic changes in folate and B12 deficiency?

A

megablastic, macrocytosis, decreased Retic index, hypersegmented neutrofils, increased biliruin and LDH

293
Q

Extravascualr hemolysis

A

done by macrophages in RE system of spleen

294
Q

Dendritic cell mechanism

A

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
Q

Partial pressure in tusse

A

40 mmHg

296
Q

why is aplastic crisis dangerous for hereditary spherocytosis?

A

severe drop in hct due to shortened RBC lifespan

297
Q

lymph flow in lymph node

A

entry in periphery, through subscapular sinus through middle of node and out of hilum

298
Q

Clonal delection

A

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
Q

Flow Cytometry

A

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
Q

why is aplastic crisis dangerous for hereditary spherocytosis?

A

severe drop in hct due to shortened RBC lifespan

301
Q

Hemolysis in SCD

A

creates more free Hb and inhibits NO release and vasodilation; also increases erythroid arginase to degrade arginine and precursor to NO.

302
Q

Treatment of Beta thal

A

transfusion to maintain 9-10 mg/dl, induction of HbF, bone marrow transplant

303
Q

HbA2

A

2% of normal HbA; functions like HbA1, but more heat stable and slightly higher oxygen affinity.

304
Q

Lymphocytes - immuno

A

T and b cells; prominent WBC in children; 20-40%

305
Q

C1-esterase inhibitor

A

regualtes the actiation of C3 so complement pathway is not always activated

306
Q

Relaxed hb

A

loosey goosy hb, as binding to oxygen increases salt bonds are brokena and affinity increases

307
Q

Band

A

more than 1/2 dimeter of nuclear indentation

308
Q

INF gamma

A

inhibits erythroid proliferation and decrease RBC production due to chronic infection and inflammation

309
Q

G-6PD Def mechanism

A

deecrased production of NADH, reduction in glutathione, and protecton from hydrogen peroxide.

310
Q

Folate and Vitamin B12 do_

A

support proliferation and maturation of RBCs, methionine sysntehsis, purine and pyrmidine biosynthesis, production of thymodylate for DNA synthesis

311
Q

oxidation of Hb_ from G6PD deficience

A

denatures Hb so it attaches to membrane to create heinz bodies, trapping/deformability and extravascualr hemolysis

312
Q

what happens with stress to reticulocytes

A

early release by 5-7 days

313
Q

Trends of Lymphocytes over lifetyme

A

1-3 days 15-30%; 4-14 days 4-18%; 15 days to 2-12 years 2-14%; 13 + 2-11%

314
Q

Pro-Bcell

A

just heavy chain with VDJu

315
Q

IgM (classes)

A

immunization to activatio of complement, V=10, but binds to 2. vicsous, only ab made in fetus

316
Q

Gower 1

A

Z1 E2

317
Q

Hb Koln

A

mutation in beta chain, physican unstable varian. Has increased oxygen affinity.

318
Q

Hb SC concentrations

A
319
Q

Fab

A

top part of one heavy and one light chain

320
Q

Methods for SCD testing

A

Sickle Dex, HB separation, isoelectric focusing, HPLC

321
Q

Chronic inflammatory disease cause anemia

A

rheumatoid arthritis, systemic lupus erythematosis, rheumatic fever

322
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

323
Q

IgA (class)

A

made by plasma cell in mucus membrane; secretory component, small amoutn of monomer in IgA plasma

324
Q

isosthenuria

A

urinary concentratin defect

325
Q

Hemolytic anemais

A

Hereditary spherocytosis, enzyme disorders, autoimmune hemolytic anemia

326
Q

adverse effects of iron overload

A

hepatic fibrosis and cirrhosis, endocrinopathies, cardiomyothpathy and sudden death

327
Q

Treatment of folate deficiency

A

1mg/day orally or parenterally

328
Q

How does 2,3- BPG concentration affect oxygen affinity

A

stabalizes T conformation and leads to decreased oxygen affinity

329
Q

Trends of Monocytes overtime

A

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
Q

when is EPO used?

A

when there is an absolute deficiency or decrease of EPO out of proportion to hct

331
Q

IgA (class)

A

made by plasma cell in mucus membrane; secretory component, small amoutn of monomer in IgA plasma

332
Q

Germinal center

A

sites of intense lymphocyte division (b cell). Increase cytoplasm compared to nucleus

333
Q

Hydroxyurea and complications

A

50% reduction in acute pain events, no evidence for chronic organ injury

334
Q

Tolerogen

A

antigen that does not ellicent and immune response

335
Q

how much can bone marrow production increase

A

6-8 fold

336
Q

Trends in basophils

A

always 0-2%

337
Q

Mekagaryopoiesis

A

Thrombopoesis. Megakaryblast matures to mekakarycoyte –> platelet.

338
Q

When does Fetal hemoglobin predominate

A

after 8 weeks gestation.

339
Q

Spleen anatomy

A

Red contains phagocytic cells to make RBC and filter RBCs; white: lymphocytes

340
Q

Risks in splenectomy

A

sepsis 1) vacciantion before, prophylatic antibiotidies, see doctor if fever above 38.5

341
Q

Saturation of heptoglobin

A

due to increased hemolysis and leads to oxidation to methemglobin

342
Q

Clonal delection

A

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
Q

Where in the body is it acidic?

A

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
Q

Polychromasia

A

premature release of reticulocytes from marrow - have bluish tint

345
Q

Beta0 thal NBS

A

F

346
Q

G-6-PD deficiency

A

X-linked recessive; prevalent in tripical eastern hemisphere, selective resistance to malaria

347
Q

NK cells

A

recognize basence of normal surface molecules

348
Q

Barts Hb

A

Y4

349
Q

cortex in llymph node

A

tightly but motile lymphocytes in follicles

350
Q

Sideroblastic Anemia

A

imparied production or protoporpyrin or incorporation of iron

351
Q

Platelet Counting range

A

6-36 fl

352
Q

when does fetus make igG?

A

3 months old

353
Q

Basophils - immuno

A

acidic cells, release histamine, Mast cells in tissue; 0.5-1%

354
Q

Acute complications of SCD

A

Vaso-occlusive pain, acute chest syndrome, infection, spelnic infarction and sequestration, stroke

355
Q

Lab/clinical findings of folate and B12 deficiency

A

megaloblastic anemia

356
Q

Fecal urobilinogen can go through three pathways

A

1) fecal mater, 2) kidney for excretion 3) EHC recycling

357
Q

measureable components of CBC?

A

Hb, Hct, RBC count, MCV, WBC count, Platelet count, mean platelet volume

358
Q

Protein calorie malnutrition laboratory/clinical

A

variable anemai, normochromic and normocytic

359
Q

Cooperative binding

A

creates a sigmoidal curve and allows for binding in lungs and release at tissue.

360
Q

Beta Thalassemia lab findings

A

decrease MCV, increased RBC, MCV/RBC

361
Q

Cathelicidin

A

bind to negative charged pathogens and bore holes - located on skin/mucosa

362
Q

Bohr effect in Fetal hemoglobin

A

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
Q

Cellular response with folate and Vit b12 def

A

increase cell size, arrest in S phase of mitosis. Cells are degraded and have ineffecitve erythropoiesis.

364
Q

Aplastic crisis

A

associated with parvovirus B19; causes low retic, severe anemia, pallor; rahs on arms and face

365
Q

Hemoglobin Chesapeak

A

High affinity Oxygen Hb due to a single point mutation; cauese reduced delivery to tissues and increase EPO release and increased RBC production

366
Q

what amino acids are on outer surface of hb

A

lysine, arginine, glutamic acid to keep hyrophilic

367
Q

where does heme bind to alpha globin

A

87th amino acid

368
Q

Rag recombinase

A

enzyme responsible for looping out random parts of DNA to greate VDJ recombination

369
Q

symptoms for PK deficiency

A

variable anemia, hemolysis, splenomegaly, gallstone, aplastic crisis

370
Q

Transfusion above 10 g/dl

A

dangerous cause hyperviscosity

371
Q

Hydrops fetalis

A

4 deletions, heart dtrain due to decreased blood volumne; subcutaenous edema

372
Q

Treatment of Renal insufficiency

A

EPO and treatment of co-morbid conditions

373
Q

Beta+ thal NBS

A

FA

374
Q

Metamyelocyte

A

must be half diameter, nuclear indentation

375
Q

what causes RBC death?

A

decreased enzymatic activity, oxidative injury, change in Ca balance, chagnes in membrane carb, antibodies, surface constituents

376
Q

PAMPs

A

pathagen associated molecular patterns, recognize forein structures through interaction with PRR

377
Q

Too many lymphocytes

A

lymphocytosis

378
Q

symptoms of G6PD deficiency

A

acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies

379
Q

Chronic Complications of SCD

A

Sickle lung disease, pulmonary hypertension, sickle nephropathy; retinopathy, leg ulcers; avascular necrosis

380
Q

lab finding for hereditary spherocytosis

A

increased retic count, decrased MCV, spherocytes

381
Q

Negative aspects of direct current technology

A

counts all cells, so includes white cells. Can not discriminate aggregated plateltes, so platelets could be artificially low

382
Q

Where does curve lean towards in gel test for Ab and AgWhere does curve lean towards in gel test for Ab and Ag

A

Ab (they are larger)

383
Q

lab tests for PK def

A

mild to severe anemia, increase retic, no specific morphology

384
Q

Hb SB+ Concentrations

A

25-45 A1; 3% A2; 2-30 HbF; 55-75 HbS

385
Q

What is in urine during increased intravascualr hemolysis

A

hemosiderin, hemoglobin, methemglobin

386
Q

how to do reticulocyte count?

A

Flow cytometry

387
Q

Affinity Maturation

A

with each replication CDR varies, if antibody encounters antigen it stays in the game.

388
Q

lab tests for PK def

A

mild to severe anemia, increase retic, no specific morphology

389
Q

Epitope

A

region of antigen that interacts with CDR, discontinous or continous, falt, also called antigenic determinants. Interacts with CDR non-covalenly.

390
Q

C1-esterase inhibitor

A

regualtes the actiation of C3 so complement pathway is not always activated

391
Q

Cellularity

A

White spaces - hematopoeitic spaces (100-age). Celularily decreases with age

392
Q

Most severe sickle cell disease

A

SCD and SB0

393
Q

Hemoglobin amount - SCD with C

A

10-12 mg/dl

394
Q

Treatmetn of Lead intoxication

A

chelation of lead

395
Q

Biochemical test for B12 deficiency

A

Increases erum folate, increasd serum methylmalonic acid, increas serum-2-methyl ctiric acid

396
Q

inheritance of hereditary spherocytosis

A

75% AD, 25% AR

397
Q

Mast cell fromation

A

requires SCF or come from the basihilic lineage with IL3

398
Q

PRR

A

pathogen recognition receptors are on surface or inner membrans (TLR)

399
Q

Classical pathway of complement

A

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
Q

Clonal Selection theory

A

cells make one antibody, choice is randome dependent on outside signals.

401
Q

Immature B cell

A

cIgM and surface IgM; clonal deletion

402
Q

NK cells

A

recognize basence of normal surface molecules

403
Q

Dendritic cell mechanism

A

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
Q

Treatment of Endocrine Disorders

A

Hormone replacement

405
Q

What contributes to antibody diversity

A

recombination, exonucleases, terminal deoxynucleotidyl transferase added random NT to N region

406
Q

Isoeletric Focusing

A

like electrophoryesis; pH gradient imbedded in gel and migrates to isoelectric point

407
Q

blood in lymph node

A

entry and exit through hilum

408
Q

Splenic Function

A

clearance of intravascualr particles; adaptive immune resonse: origin of igM

409
Q

IgD (class)

A

B cell recptor antibody (antibody forming cells)

410
Q

What is % sat at 30 mmHg

A

60%

411
Q

Renal insufficiency in Anemia

A

no kidney function means no EPO, so no erythroid proliferation and decrease RBC production

412
Q

lab finding for hereditary spherocytosis

A

increased retic count, decrased MCV, spherocytes

413
Q

SB0 - NBS

A

FS

414
Q

Intravascular Hemolysis

A

breakage of RBC in vascualr system

415
Q

why is myoglobin not a good oxygen carrier

A

high affinity for oxygen at low oxygen concetnraitons - can’t release at tissues.

416
Q

Cold

A

IgM (some IgG) bind in extremitie and return in warmth to acivate complement and intravascualr hemolysis

417
Q

Maturation of T lymphocyte

A

T lymphoblast

418
Q

IgD (class)

A

B cell recptor antibody (antibody forming cells)

419
Q

>30% methemoglobin

A

fatigue, lightheaded, headache

420
Q

Partial pressure in lungs

A

100 mmHg

421
Q

which has neurological features B12 or folate?

A

B12 - sensory loss, proprioception loss, ataxia, gait distrubances, Babinski reflex, cognitive and emotional changes.

422
Q

Lab finding of AIHA

A

Increased Retic, increased bilirubin, hemoglobinuria, warm + Dat for igG or Cold + Dat for complement

423
Q

INFbeta

A

Triggerd by Malignancies and sepsis, to inhibit erythroid proliferation and decrease RBC production

424
Q

Why does transfusion help with beta thalassemia

A

supresses inate RBC production and marrow expansion

425
Q

igG (classes)

A

most abundant, passes placent to fetus, longer effect after immunization, half life of three weeks, opsonizing, 2 igG to activate complement

426
Q

Lectin pathway

A

complement pathway; mannose bidning protein binds to bacterial structures to activate Complement pathway MBP- 4-2-3-5-6-7-8-9

427
Q

Howell-jolly bodies

A

basophilic nuclear remnants in erthyrocytes - due to damaged or absent spleen

428
Q

what is %sat at 40 mmHg

A

75%

429
Q

chronic infections that cuase anemia

A

pulmonary abscesses, meningitis, chronic GU infection

430
Q

how long in storage pool for granulopoiesis

A

5-7 days beore going into vessles walls in marginated pool or circulation in peripheral blood.

431
Q

Hemoglobin amount - B+ Thal

A

11-13 mg/dl

432
Q

How does mRNA for antibody form?

A

splicesome splices out primary RNA to make VDJ u or VDJdelta

433
Q

Too many monocytes

A

monocytosis

434
Q

Cholelithiasis

A

gallstones due to increased bilirubin, –> obstruction

435
Q

lymph flow in lymph node

A

entry in periphery, through subscapular sinus through middle of node and out of hilum

436
Q

which deficiency is faster, folate or B12?

A

Weeks to months for folate, very slowly for B12 (years)

437
Q

Acute Chest Syndrome

A

Rib/vertebral infarction, infection, bone marrow embolysn lead to decreased blood O2, chest pain, fever, decreased hemoglobin

438
Q

IgG Serum

A

1000 mg/dl; lightest at 150,000 D

439
Q

is serum creatinine a good indiciator of kidney function?

A

no - can have widely different GFR

440
Q

mature B cell

A

sIgM and sIgG with same specificity

441
Q

Treatment of Structural Hb Variants

A

folic acid, splenectomy not curative, no blood transfusion

442
Q

Endocrine disorders that cuase anemia

A

thyroid, adrenal insufficiency

443
Q

Peyer Patch

A

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
Q

clinical features of Lead intoxification

A

personality changess, irritability, headache, weakness, ab pain, vomiting

445
Q

Underproduction or normla Hb in thalassemia

A

Small RBC, low hb concentration, uniform RBC, Increased RBC production

446
Q

Chelation therapy

A

deferasirox, deferoxamine, deferiprone

447
Q

Lymphopoiesis

A

vast majority in Bone Marrow; T cells mature in thymus. Lymphoblast –> lymphocyte

448
Q

Where in the body is it basic?

A

CO2 is exhaled in the lungs and leads to increased oxygen affinicity

449
Q

Pre-B cell

A

cytoplasmic IgM (light and eavy)

450
Q

Basophils in Differential

A

neuclus is obscered by coards, purple, black granules

451
Q

MCHC

A

Mean Corpsular Hemoglobin Concentration; avg conc of hg in given volume of red cells

452
Q

blood in lymph node

A

entry and exit through hilum

453
Q

cortex in llymph node

A

tightly but motile lymphocytes in follicles

454
Q

HbD

A

punjab; Beta 121 Glu –> Gln (polar); india, packistan, iran

455
Q

TLR

A

bind to initiate inflammatioon cascade and ultimately activate NFkB

456
Q

how long do nutrophils spend in periphery

A

less than 10 years, where they are taken out in urine or feces or by monocytes/macrophages

457
Q

caues of folate deficiency

A

dietary insufficiency, malabsorption, drugs and toxins, errors in folate metabolism, increased demand, increased metabolism/loss

458
Q

Bohr Effect

A

Oxygen is held tightly in basic conditions and released in acidic

459
Q

Gamma vs Beta Globin

A

Differs by 39 amino acids; binds to 2-3 BPG poorly, so it is stabalied in R state and shifts curve to left

460
Q

supportive care, avoid oxidant drugs/food, folate, transfusion if severe

A

antibodies that cause hemolysis intravascualr and extravascualr

461
Q

30-50% methemoglobin

A

moderate depression of CNS, achycardia, tachypnea, shortness of breath

462
Q

here does heme bind to beta globin

A

92nd amino acid

463
Q

Treatment of B12 deficiency

A

1mg injections weekly/monthly or 2ug orally 2x day

464
Q

Cooley’s Clinical manifestations

A

Bone marrow expansion - hair on end, spelenmegaly, osteopenia, iron overlad

465
Q

igG (classes)

A

most abundant, passes placent to fetus, longer effect after immunization, half life of three weeks, opsonizing, 2 igG to activate complement

466
Q

methyltetrahydrofolate

A

is transferred to liver for sotrage, tissue, and undergoes EHC recycling with jejunum

467
Q

Spleen anatomy

A

Red contains phagocytic cells to make RBC and filter RBCs; white: lymphocytes

468
Q

Risks in splenectomy

A

sepsis 1) vacciantion before, prophylatic antibiotidies, see doctor if fever above 38.5

469
Q

Sickle cell trait Concentrations

A

55-60 A1;3% H2;

470
Q

Normocytic anemia types

A

anemia of chronic disease, sickle cell

471
Q

Polymorphonuclear cells

A

eosinophils, basophils, neutrophils

472
Q

Allotypic exclusion

A

like X inactivation of antibodies, recombination can be done if initial antibody is unsuccessful

473
Q

Class switching

A

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
Q

Anemia of Chronic Disease

A

Chronic infection, chronic inflammation, malignant disease, renal insufficiency, lead intoxification, endocrine disorders

475
Q

Too few lymphocytes

A

lymphopenia

476
Q

Pre-B cell

A

cytoplasmic IgM (light and eavy)

477
Q

Lab findings of Chronic infection, inflammation, malignancy

A

mild/moderate anemia, normochromic/microcytic, dec serum Fe, dec TIBC, normal to increase ferriting, decrease EPO and dec Retic count

478
Q

Retic count - SCD with B0 Thal

A

5-30%

479
Q

Domains in antibodys

A

made of 7 Beta sheets for rigidity. The region sin between the sheets form the variable regions. Held together by disulfide bonds

480
Q

laboratory features of renal insufficiency

A

mod/severe anemia, normochromic, normocytic, EPO dificiency

481
Q

Neutrophils - immuno

A

fasts moving and move prominetn cell sin adults 40-60%

482
Q

Perinuclear hof

A

golgi in myelocyte

483
Q

IgE (class)

A

binds to mast cells and eosinophils, triggers histamine release for allergic reactions; parasite killer

484
Q

Retic count - SCD

A

5-30%

485
Q

Pathophysiology of SCD

A

hemolysis, increased endothelial-1, proliferation of smooth muscle, increased adhesion, increased platelet activation

486
Q

Neutrophils - immuno

A

fasts moving and move prominetn cell sin adults 40-60%

487
Q

MCH calculation

A

Hb/RBC count

488
Q

how type of iron binds to Hb?

A

Ferrous 2+

489
Q

Methemoglobin

A

Ferric form binds to Hb instead of Ferrous. Capacity to carry oxygen is reduced and curve shifts LEFT?

490
Q

Classical pathway of complement

A

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
Q

Newborn screening SS

A

FS

492
Q

Mast cells

A

Found in tissues rather than peripheral blood, finer granules with one prominent nuclues.

493
Q

Transfusions

A

dilute out sickle RBC and provides healthy RBCs

494
Q

acute anemia, hyperbilirubinemia, hemolysis, reticulocytosis, blister cells, bite cells, heinz bodies

A

supportive care, avoid oxidant drugs/food, folate, transfusion if severe

495
Q

where do dendritic cells gather in lymph node

A

between cortex and paracrotex

496
Q

where do dendritic cells gather in lymph node

A

between cortex and paracrotex

497
Q

Alternative complement pathway

A

cell wall structures in microbes activate C3 directly in absense of antibody (innate)

498
Q

Papin

A

reduces disulfide bonds to create 2 Fab and 1 Fc

499
Q

Moya Moya fromation in SCD

A

abnoraml cerebral blood vessesl due to vasculopathy

500
Q

IL-1

A

stimualted by chronic infection and inflammation to decrease iron and decrase erythropoietin