Exam 1 Flashcards Preview

Blood and Lymph > Exam 1 > Flashcards

Flashcards in Exam 1 Deck (500):
1

Positive cooperativity

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

2

Acute chest syndrome associated complications

kidney and liver failure

3

How does temp influence saturation curve?

shifts to the right

4

Vitamin B12 absorption

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

5

Thrombopoetin

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

6

Treatment of Chronic infection/inflammation/malignancy

treatment of underlying disease to decrease cytokines and interluekins

7

Redicualte in circulation

1-2 days

8

Idiotype

difference in CDR due to specificity.

9

Pro-Bcell

just heavy chain with VDJu

10

Calculated Hematocrt

RBC * MCV

11

Alpha globin in DNA

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

12

post-capillary venules in lymph node

high-culoidal to react to lymphocytes

13

too few monocytes

monocytopenia

14

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

15

IgD

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

16

Hb Portald 1

Z2Y2

17

RDW calculation

standard deviaiton of values of MCV

18

TLR

bind to initiate inflammatioon cascade and ultimately activate NFkB

19

Allotypic exclusion

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

20

Hemolytic anemais

Hereditary spherocytosis, enzyme disorders, autoimmune hemolytic anemia

21

Fe Deficient - Concentrations

99% a1; 2% A2;

22

MCV

Mean Corpsular Volume, average volume of RBC

23

Spectrin, ankryn, band 3 alll..

lead to destabalization of lipid bilayer and microspherocytes --> deformability

24

How does temperature affect oxygen affinity

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