Cumulative From Exam (I-II-III) Flashcards
(247 cards)
1
Q
What is the disorder when an enzyme is missing in heme synthesis?
A
2
Q
Multi fill in the blank - Aplastic anemia+ hemolytic anemia (lab findings)
A
3
Q
Deficiency disorder hemostasis RBC+WBC
Propitious anemia B12 , Intrinsic factor
A
4
Q
APTT - monitors heparin
A
Alternative anti factor Xa - Assay
5
Q
PT - Monitors Warfarin/Coumadin
A
Withholds vitamin K form prothrombin proteins
6
Q
average blood volume
A
4-6 L in adult
7
Q
blood pH
A
7.35-7.45
8
Q
blood specific gravity
A
1.049-1.065
9
Q
blood composition
A
55% plasma/serum (fluid)
45% formed elements (cells)
10
Q
Serum
A
NO anticoagulant used, some clotting factors consumed
11
Q
In serum, there are proteins …
A
55% are albumin
12
Q
WBC count
A
4.8-10.8x10^3
13
Q
Major blood functions
A
Transport oxygen to cells
Transport carbon dioxide and wastes away
Provide defense
Regulate body pH , body temp, fluid balance
14
Q
homeostasis
A
maintaining a constant environment equilibrium
(major blood function)
15
Q
Platelets (thrombocyte)
A
Size: 1-4 um
No nucleus
Life span: 10 days
Function: Hemostasis –> blood clots, and arrested bleeding
16
Q
Neutrophils
A
Inflammation and phagocytosis - 1st line of defense against infections.
2-5 lobes connected by thin filament, segmentation allows passing through openings between lining cells
life span: 6-10hrs
17
Q
Eosinophil
A
Defense in parasitic and fungal infections.
Secondary granules (red/orange)
18
Q
Basophil
A
Mediates allergic reactions
smallest WBC in circulation
19
Q
histamine
A
vasoconstriction
20
Q
heparin
A
blood thinner
21
Q
lymphocyte functions
A
Immune response and viral infections
varies in size and shape
22
Q
Monocytes function
A
Phagocytosis
Nucleus: horseshoe, lima bean, convoluted
Cytoplasm: Ground glass - cloudy
23
Q
mature RBC - Erythrocyte
A
Major function: Oxygen transport from lungs to tissue
6-8um Biconcave disc –> max SA for gaseous exchange
No nucleus - no mitosis
Hgb is 95% dry weight
120 days life span.
24
Q
Organs within Hematopoietic system
A
BM
Liver
Spleen
Lymph node
Thymus
25
Embryo
Mesoblastic phase
26
Fetal
Hepatic phase
27
Birth
Meduallry phase
28
Embry- Mesoblastic phase
2 weeks - 2 months gestation
**Yolk sak - mesoderm layer**
Hgb Formed
- Hgb Gower 1
- Gower 2
- Hgb Portland
Embryonyc Hgb^
29
Fetus - hepatic phase
2 months - 7 months
Liver and spleen
Hgb Formed:
- Fetal hemoglobin
- Hemoglobin A1 (small amount)
30
Birth- medullary phase
7 months - rest of life!
BM = intramedullary hematopoiesis - red marrow
Hgb Formed
- Hemoglobin A1
- Hemoglobin A2
31
Infancy and childhood
Volume and red marrow in infacts = red marrow in adults (distal long bones and axial skeleton)
at 4 years, yellow marrow, starts to replace red marrow --> limits sites of hematopoisis
32
active hematopoietic sites in axial skeleton
Sternum
Ribs
Pelvis
Vertebrae
Skull (after 40, no more skull)
33
Pelvis and sternum are
preferred bone marrow aspiration sites --> last place to be replaced by yellow marrow
34
Blood cell development theory
**Monophyletic** --> All blood cells arise from one precursor cell that is multipotential or pluripitenital and called stem cell
35
pluripotential/multipotential
can develop into any cell line in the body
36
progenitor cell
committed to cell line
37
cytokines
soluble messages to tell early cells to differentiate
38
Thrombopoietin
stimulates CFU-Meg, and causes release of platelets
39
Erythropoitetin
Stimulates CFU-E, and regulates erythroid progenitor cells to mature.
Glycorpotein prodcued by the peritubular interstitial cell in juxtaglomerular apparatus (kidney tubulus)
40
T cell
Cytotoxic, suppressing actvites
41
B cell
antibody production, can futher develop to plasma cell and secrete antibodies
42
CFU-GEMM
gives rise to a serius of committed cells (MYELOID)
43
BM
Myeloid: Eryrhoid ratio
2:1 - 5:1
44
Eythrpid maturation
Large --> small/**pyknotic** (condensation and shrinkage through degeneration)
Euchromatin --> heterochromatin --> Nucleus is extruded
45
Cell development: Asynchrony
Megaloblastic anemia
46
Basophilic Normoblast/prorubricyte
Chromatin: Some **granularity**
Cytoplasm: Slighlty less basophilic "**Cornflower blue**"
47
Polychromatophilic normoblast
Chromatin moderately compacted, condensed, "soccer ball"
48
Orhtochromatic normoblast (nRBC)
Last nucleated stage
Chromatin: **completeley pyknotic**
49
Polychromatiophilic erythrocyte
**NO NUCLEUS**
polychromatophilia
50
Wrights stain
polychromatiphilic erythrocyte
51
new methylene blue (vital stain)
reticulocyte
52
mature erythrocyte
central pallor
no longer able to synthesize hemoglobin
53
RBC membrane
selective barrier
Water and anions - passive diffusion
cations and others - active transport
54
potassium
found inside RBC 25:1
55
sodium is found
outside RBC 1:12
56
Outer hydrophilic portion of RBC membrane
Containts glycolipids, glycoproteins, proteins
57
central hydrophobic layer
contains proteins, cholesterol, phosphilipids
58
inner hydrophilic layer
protein
59
cholesterol
Central hydrophobic layer
- Target cells
- Acanthocytes
60
Choline phosphilipid
phosphatidyl choline and sphingomylein
outer half
61
amino phospholipids
phosphatidylethanolamine and phosphatidyl serine
inside
if outside, can initaite clotting
62
integral protein example
glycophorin
63
peripheral membrane pritein
spectrin, beneath lipid bilayer, forms cytoskeleton
64
Glycophorin
Principle RBC integral membrane protein
**sialic acid**
65
spectrin
most abundant peripheral membrane protein
66
RBC deformability
RBC survival through microvasculature
Oxygen deliver function
67
hgb infant
14-22g/dl
68
hgb male
14-18g/dl
69
hgb female
12-16g/dl
70
Hemoglobin =
4 heme rings
4 iron
4 globin chains
71
protophoryns
precursor of heme
72
1 heme ring =
1 porphoryin = 4 pyrrole rings
73
major rate limiting step in heme syntehsis
ALA synthetase
74
HgbA1
2a + 2 b
75
Hgb A2
2a+2d
76
HgbF
2a+2y
77
methemoglobin
Iron is oxidied to ferric state (Fe3+)
78
carboxyhemoglobin
Oxygen is replaced by CO - increased binding
79
sulfhemoglobin
sulfur incorporated into heme structure
irreversible
80
90% of RBC energy comes from
non-oxidative pathways
81
RES
Removes RBC from circulation
- **spleen**
82
spleen
primary site of phagocytosis
home to littoral cells
83
Extravascular hemolysis
Heme --> biliverdin --> unconjugated bilirubin --> conjugated bilirubin --> urobilinogen
Urobilinogen eliminated in stool
84
Hgb released in blood vessel
Circulates - Hemoglobinemia
- Filtered at the kidney = hemoglobinuria
Carried by haptoglobin to liver
85
if BM cannot keep up
Extramedullary hematopoiesis
86
renal failure patients
lack erythropoietic response to hypoxia
87
female RBC
4.2-5.4 x 10^6 ul
88
Male Rbc
4.7-6.1x10^6 ul
89
HCT
Proportion of red blood cells in whole blood
90
MCV
80-100fl
91
MCH
average Hgb mass/weight
27-31pg
92
MCHC
32-36 %
Average Hgb Concentration
93
neutrophil %
50-70%
94
lymphocyte %
20-44%
95
monocyte %
2-9%
96
band neutrophil
2-6%
97
eosinophil
0-4%
98
basophil
0-2%
99
Microcyte
diameter <6um
MCV<80
100
normocyte
diam : 6-8um
MCV: 80-100fl
101
anisocytosis
any significant variation in size
102
macrocyte
diam: >9
MCV:> 100 fl
103
microcyte cause
lack hemoglobin synthesis elements
104
Macrocyte
impaired DNA synthesis
Megaloblastic Anemia (B12 deficiency)
105
Myeloblast
no granules present
106
Promyelocyte
Primary granules, CONTAINING Myeloperoxidase
107
eosinophilic myelocyte
Filled with large red/orange secondary granules
108
eosinophilic metabmyelocte
indention = less than half nuclear diameter
109
Eosinophilic band
indention: >1/2 diameter
110
Eosinophil - last stage
Bi lobed
Red orange secondary granules
111
Basophilic myelocyte
filled with large purple/black secondary granules
112
Dawn of neutrophilia
first distinguishable neutrophil
113
Myelocyte --> metamyelocyte -->
band --> segmented
114
primary granules
starts in promyelocyte stage
All granulocytes = non specific
115
primary granules
specific for granulocytic line
116
promonocyte
peaked crease
117
tart cell
phagocytic cell engulfing at least the nucleus of another cell
118
liver
kupffer cell
119
lungs
alveolar macrophages
120
Bone
osteoclasts
121
skin and mucosa
langer hans
122
Thrombopoiesis unique
cell volume increases
123
megakaryoblast
Pseudopodia present
124
promegakaryocyte
pseudopodia
fine azurophilic granules
125
hyalomere
pale portion
126
lymphoblast
intense staining at periphery
127
Target cell
increased surface to volume ratio
128
stomacyte
mouth like
BRC membrane cation permeability
129
ovalocytes seen in
Megaloblastic anemia
IDA
130
sickle cell (drepanocytes)
Hemoglobin S
131
helmet cells (Bite cells)
"pitting"
132
G6PD defieicny
Heinz bodies
133
Howell jolly bodies
DNA remnants
134
Basophilic stippling
RNA and mitochondrial remnants
135
wright stain
pappenheimer bodies
136
Prussian blue stain
sideritic granules
137
rouleaux
stack of coins
lowering zeta potential - cells become sticky
138
pelter huet anomaly
decreased lobulation
139
Dohle Bodies
RNA remnants
140
Auer Tods
Needle like bodies
fusion of primary granules
141
Barr body
inactivated X chromosome
142
microcytic, hypochromic anemia
Hgb synthesis defect
microcytic: <6 um
MCV<80
Hypochromic: CAP>30
MCHC<32
143
Anemia definition
Deficiency of oxygen delivery to the tissues / healthy red blood cells
Low RBC count
Low hematocrit
Low hemoglobin
144
Deficiency of iron
IDA
145
Abnormal heme synthesis
porphoryias
146
Abnormal globin chain synthesis
thalassemia
147
Abnormal utilization of iron
Anemia of chronic disease
Sideroblastic anemia
148
heme iron
complexed into a porphyrin ring to form heme ring of hemoglobin
149
Transferrin
Carrier protein for serum iron
150
Ferritin
Storage in BM and liver, easily mobilized
151
Hemosiderin
Lysosomal membrane of macrophages, long term storage
152
serum iron range
50-150 ug/dl
153
TIBC
250-450
154
Transferrin % saturation
20-50
155
Serum ferritin male and female
M: 20-250
F : 10-120
156
IDA cause - most common in western world
Blood loss
- mens: female
- GI bleed: male
157
pica
abnormal cravings
158
pagophagia
craving for ice
159
geophagia
craving for dirt, clay
160
koilinychia
spoon nails
161
IDA lab findings
microcytic, hypochromic RBC
Ansisopoikilocytosis
162
IDA
DECREASED SERUM FERRITIN --> found in all IDA stages
Increased TIBC
163
Most common anemia found in hospitalized patients
ACD
Low serum iron, Normal or High Serum ferritin, Decreased TIBC
164
ACD lab finding
N or Increased M:E ratio
due to decreased erythropoiesis
hemosiderin present
165
ACD treatment
Corrrection of primary disease state
166
SA lab findings
Dimorphic population
- Microcyte, normocyte
- hypochromic, normochromic
anisopoikiliocytosis
pappenheimer bodies
167
SA LAB FINDINGS
Increased Serum ferittin
Normal or Low TIBC
Increased Serum Iron
Increased Transferrin saturation
168
SA BM
Ringed sideroblast.
Erythroid hyperplasia
169
hemochromatosis
tissue damage resulting from excess iron
170
Heme synthesis issue
P8 deaminase
Prophobilinogen accumulation
171
hemoglobinopatheies
qualitative or quantitative abnormalities in the synthesis of hemoglobin
172
Thallasemia
Quantitative abnormalities resulting in decrease in production of globin chain synthesis
173
definition of thalassemia
group of disorders that result in a decreased production of globin chains in hemoglobin usually resulting in a microcytic, hypochromic anemia
Alpha -> defect in alpha chain
beta --> defect in beta chain
174
hemoglobin Bart
4 gamma chains
175
Hemoglobin H
4 beta chains
176
types of alpha thalassemias
Hemoglobin bart and H
177
Alpha a thalassemia
hemolysis
178
beta b thalassemia
ineffective erythropoiesis
179
Beta (B) Thalassemia
Clinically seen 1st year of life
Forms HgbF and HgbA2
180
B+
gene produces a reduced amount of beta chains
181
BB
no anemia
182
Beta thalassemia minor
BB0,BB+ (trait)
183
Beta thalassemia intermedia
B+B+ (less severe B+ types)
184
B thalassemia Major
B0B0, B+B+. ,B0,B+
185
B thalassemia major pathophysiology
bone changes (skull, long bones, hand)
Hepatospleenomeglay
186
untreated B thalassemia major
Massive hepatosplenomegaly --> due to extramedaullar erythropoiesis
187
treatment problems with B thalassemia major
iron overload
188
B thalassemia major peripheral smear
Microcytic, hypochromic
Anisocytosis, poikilocytosis
Target cell
Howell jolly body
189
alpha genes
chrom 16
190
aa0, a+a+
a thalassemia minor
191
Hemoglobin H disease
a0a+
192
Hemoglobin bart (4 gamma)
a0a0
193
Hemoglobin H disease
5-40% Hgb H (4 beta chains)
194
Hemoglobin Bart
4 gamma chains
Lethl disease
4gamma chains
195
Thalassemia findings
Microcytic, hypochromic
- reticulocytosis
- poikilocytosis - elliptocytes, target cells, fragments
LOW CBC
normal/high Iron
196
Thalassemia, decreased osmotic fragility
due to higher surface to volume ratio
197
Sickle cell
Hemoglobin S
A2B2 (6Glu-Val)
198
beta 6th position
Glutamic acid --> valine
199
SS anemia
Normocytic, Normochromci
Vasoocclusions --> Ischemic tissue injury
200
SS - aplastic
Decreased reticulocyte count.
201
SS - hemolytic
Increased Reticulocyte count
202
SS- vaso-occlusive
occlusions of small blood vessels --> tissue damage and necrosis
203
SS anemia - acute chest syndrome
2nd most common cause of hospital admission in SS
Occlusion in the pulmonary vasculature
204
Dactylitis
painful swelling of hands and feet
205
cutaneous manifestations
development of ulcers and sores, often on lower legs
206
SS anemia, hand-foot syndrome
microinfraction in small bones, of hands and feet lead to unequal growthand bones feformities
Fingers and toes
207
SS stroke
ischemia or hemorrhagic lesi0on in specific cerebral vessel
208
hydroxyurea
increases Hgb F production
209
Aplastic crisis, only one with
decreased reticulocyte count
- sickled cells, (drepanocytes), oat cells
Howell jollu
Target cells
Schistocytes
210
Reduced HGb S is insoluble
turbid/opaque suspension
211
Sickle Cell anemia
>80% S
<20% Hgb F
TIny A2
212
SS trait
60% Hgb A 1
40% Hgb S
Tiny A2
Tiny Hgb F
213
Hemoglobin C
Glutamic acid --> lysine in 6th position
Hgb C crystals
95% Hgb C
trait: 40% hgb C
214
Hemoglobin SC disease
Hgb SC crystals
50% Hgb S (valine)
50% Hgb C (lysine)
215
Hemoglobin S disease
Microcytic, hypochromic
216
GPI anchor (RBC membrane)
PNH (piga mutation)
217
Hereditary stomacytosis
allowing Na+ into cell
218
hereditary xertcytosis
leaking K+
219
HPP
Spectrin (assembly issue)
Schisocytes
220
HE
Elliptocytes (spectrin)
Defective/deficient
221
Hereditary Spherocytosis
Spherocytes (spectrin), most common
Spectrin Deficiency
Loss of SA
222
Hexose monophosphate pathway
GP6D deficiency
protect Hgb
Bite cells, Heinz bodies
223
glycolytic pathway
Pyruvate kinase deficiency
non-oxidative
generates 90% of ATP needed by RRBC
Forms 2 ATP
low ATP --> Rigid RBC hemolysis
224
Methemoglobin reductase pathway
methemoglobin reductase deficiency
important in maintaining heme iron in reduced (fe2_, ferrous form)
Methemoglobin
NADH reductase deficiency
225
PNH lab finding
hemoglobinuria
226
macrocytic anemia
>100mvc
227
megaloblast
large and abnormal precursor cell in the BM exhibiting asynchrony
228
Pancytopenia BM
Aplastic anemia
Congenital = fanconi
Aquired = idiopathic 2
229
erythroid hyperplasia
congenital dyseryythropietic anemias
230
erythroid hypoplasia
diamond BlackFan anemia
231
Causes of impaired DNA synthesis (TTP synthesis inhibition)
Vitamin B12 deficiency
PERNICIOUS ANEMIA , lack of intrinsic factor
Folic acid deficiency
232
IF
glycoprotein secreted by parietal cell (stomach)
233
glossitis (b12 and folic acid )
smooth sore tongue
234
B12 deficiency - 4 Ps
Peripheral Neuropathy
Pyramidal Tract signs
Posterior spinal column degradation
Psychosis
peripheral nerves are demyelinated
235
Parestheia -=
tingling /prickling feeling
236
aplastic anemia definition
hypoproliferative disorder with cellulae depletion and reduced production of all blood cells, pancytopenia
237
cytopenia
abnormalitieis or deficiencies in specific blood cell elements
238
pancytopenia
depression of each of the normal bone marrow elements
239
Idiopathic
majority of cases of aplastic anemia
240
Aplastic anemia - BM
Dry tap
241
6, purple
EDTA, CBC, ESR
242
1st choice for vein
median cubital vein
243
antiseptic
any agent used to clean living tissue
244
torniquet must
be removed before removing needle
245
megaloblastic anemia peripheral smear
hypersegmentation
HJ bodies
Cabot Rings
Teardrop cell
246
Megaloblastic anemia gives rise to Low serum folate
Folic acid deficiency
Low serum folate
247
Magloblastic anemia VB12 deficiency
Low serum B12
Nuerological
