Schoenwald Flashcards
1
Q
symptoms of anemia
A
fatigue
tachycardia
HSM
dyspnea
pallor
bone pain
2
Q
anemia is evaluated using what 2 values
A
- size → MCV
- hemoglobin concentration → MCHC
3
Q
types of RBC morphology
A
spherocytes
schistocytes
target cells
tear drops
sickle cells
rouleaux
4
Q
what are the 3 mechanisms by which anemia occurs
A
- decrease in RBC → acute or chronic
- decreased production of RBC
- increased destruction of RBC → hemolysis
5
Q
what are the 4 mechanisms of anemia caused by increased RBC destruction
A
- external factors
- hereditary
- acquired defect
- paroxysmal nocturnal hemoglobinuria
6
Q
anemia definition
A
decrease in RBC
7
Q
what condition is caused by RBC becoming more prone to lysis dt a deficiency in CD55 and CD59
A
paroxysmal nocturnal hemoglobinuria
8
Q
paroxysmal nocturnal hemoglobinuria is an __ disease
involving a defect in the __ gene
A
acquired
PIG-A
9
Q
paroxysmal nocturnal hemoglobinuria results in __
A
venous thrombosis
10
Q
paroxysmal nocturnal hemoglobinuria involves what 4 veins
A
hepatic
portal
mesenteric
cerebral
11
Q
what is the cardinal symptom of paroxysmal nocturnal hemoglobinuria
A
episodic hemobloginuria of the 1st morning urine
12
Q
increased destruction of RBC is broken into what 3 categories
A
- external factors
- hereditary internal factors
- acquired defects
13
Q
name 3 microcytic anemias
A
- iron deficiency
- thalassemia
- anemia of chronic dz → late stage
14
Q
in thalassemia, all iron labs are
A
normal
15
Q
in IDA, __ and
__ are low,
and __ is elevated
A
low: ferritin, transferrin
high: TIBC
16
Q
in the US, what are the 2 most common causes of IDA
A
- GI blood loss
- menstrual blood loss
17
Q
outside the US, what is the most common cause of IDA
A
poor nutrition
18
Q
IDA can also be caused by (2)
A
- malabsorption
- increased demand → pregnancy
19
Q
thalassemia is caused by __
A
deficient production of Hgb
20
Q
what are the 2 causes of decreased Hgb production in thalassemia
A
- mutation
- complete loss of one or more of the 4 copies of the alpha globulin chain
21
Q
what are the 2 mechanisms by which RBC are removed from circulation
A
- extravascular
- intravascular
22
Q
extravascular removal of RBC from circulation involves __
in the __
and __
A
phagocytosis
spleen and liver
23
Q
what are 2 complications of extravascular RBC removal from circulation
A
- jaundice
- gallstones
24
Q
intravascular removal of RBC from circulation involves
A
destruction of RBC in the vessel
25
what are 3 complications of intravascular removal of RBC from circulation
1. tubular necrosis dt hemoglobinemia
2. jaundice
3. gallstones
26
what 3 lab values are increased in hemolysis
bilirubin
potassium
LDH
27
what 2 lab values are decreased in hemolysis
RBC
haptoglobin
28
in what 2 conditions will you see spherocytes
1. hereditary spherocytosis
2. immune hemolysis
29
in what 2 conditions will you see schistocytes
1. TTP
2. HUS
30
in what 2 conditions will you see target cells
hemoglobinopathies
1. sickle cell anemia
2. thalassemia
31
in what condition would you see teardrop cells
myelofibrosis
32
in what 2 conditions would you see sickled cells
1. sickle cell anemia
2. thalassemia minor w. sickle cell
33
in what condition would you see rouleaux anemia
multiple myeloma
34
what causes rouleaux
stacking of RBC due to increased globins or decreased albumin
35
what does increased reticulocyte indicate in terms of bone marrow
bone marrow is intact and able to produce RBC
36
in what 2 conditions would you see increased reticulocytes
1. acute blood loss
2. hemolysis
37
what does decreased reticulocyte count indicate in terms of bone marrow (2)
1. primary bone marrow disorder
OR
2. deficiency in building blocks
38
what are the 3 building blocks of RBC
1. iron
2. B12
3. folate
39
what 3 labs are decreased in IDA
1. ferritin
2. serum iron (+/-)
3. transferrin saturation
40
what 2 labs are increased in IDA
1. TIBC
2. RDW
41
what is transferrin saturation
ratio of serum iron to TIBC
42
what is nl for transferrin saturation
\>20%
43
what is transferrin saturation in IDA
\<10%
44
on a hgb molecule, there are __ copies of the alpha globulin chain
and __ copies of the beta globulin chain
4
2
45
in alpha thalassemia loss of one copy of the alpha globulin is called
silent (carrier)
46
in alpha thalassemia, loss of 2 copies of the globulin gene is called
trait → asymptomatic
47
alpha thalassemia trait is asymptomatic, but __ is low
MCV
48
in alpha thalassemia, loss of 3 copies of the alpha globulin chain gene is called
hemoglobin H disease (HbH) → marked anemia
49
in alpha thalassemia, loss of 4 copies of the alpha globulin chain gene is called
hydrops fetalis → stillborn
50
alpha thalassemia is prevalent in what 2 patient populations
Africans
Asians
51
does alpha thalassemia require a blood transfusion
no
52
beta thalassemia is characterized by a gene malfunction, resulting in
complete loss of the hemoglobin protein
53
beta thalassemia is prevalent in what patient population
mediterranean descent
54
what are 2 consequences of beta thalassemia when just alpha chains remain
1. hemolysis
2. ineffective erythropoiesis
55
beta thalassemia is categorized in to
major
minor
56
major thalassemia requires what type of tx
blood transfusion
57
usual onset of beta thalassemia is age \_\_
and results in \_\_
6 months
skeletal abnormalities
58
does beta thalassemia minor require a blood transfusion
no
59
what will you see in labs for beta thalassemia
disproportionate Hgb and MCV → 10/55
60
what 2 labs will be decreased in thalassemia
MCV
Hgb
61
what RBC morphology will you see in thalassemia
anisocytosis
poikilocytosis → target cells
62
what is anisocytosis
RBC of unequal size
63
what is poikilocytosis
abnormal shaped blood cells
64
name 4 conditions that can cause anemia of chronic dz
1. lung carcinoma
2. hodgkin lymphoma
3. rheumatoid arthritis
4. infxn → TB
65
anemia of chronic dz is initially \_\_
but can become __ in later stages
normocytic
microcytic
66
what lab value is increased in anemia of chronic dz
ferritin
67
what 2 lab values are decreased in anemia of chronic dz
serum iron
TIBC
68
in anemia of chronic dz, __ and
\_\_ cause erythropoietin to be decreased
TNF
interferons
69
in anemia of chronic dz, __ is stimulated,
which impairs release of iron from storage
hepcidin
70
what is hepcidin
a protein that regulates entry of storage iron into circulation
71
in IDA, __ (3) are decreased
and __ (1) is increased
decreased: ferritin, iron, transferrin saturation
increased: TIBC
72
in anemia of chronic dz, __ (2) are decreased
and __ (2) are increased
decreased: iron, TIBC
increased: ferritin, transferrin saturation
73
vitamin B12 deficiency causes __ DNA synthesis
involving \_\_
impaired
all bone marrow precursors (not just RBC)
74
impairment of DNA slows __ maturation
but not __ maturation
nuclear
cytoplasmic
75
B12 is needed to regenerate what molecule
tetrahydrofolate
76
what is tetrahydrofolate
the main active metabolite of dietary folate
77
what is the function of tetrahydrofolate (folate)
DNA synthesis and repair
78
why won't folate supplementation effectively treat B12 deficiency
it will correct the anemia but not the neurologic symptoms bc B12 is needed to regenerate tetrahydrofolate
79
aside from iron tests, what 2 elevations will you see in B12 deficiency
1. homocysteine
2. methyl-malonic acid (MMA)
80
B12 deficiency causes anemia and what other two -penias
thrombocytopenia
leukopenia
81
B12 deficiency causes ineffective \_\_
and __ deficiencies
erythropoiesis
neurologic
82
name 2 neurologic deficiencies that are seen in B12 deficiency
1. peripheral neuropathy
2. spinal cord pathology
83
are neurologic deficiencies seen in folate deficiency
no!
84
name 5 causes of B12 deficiency
1. pernicious anemia
2. nutrition
3. gastrectomy
4. ileal dz
5. diphyllobathrium latum infxn (fish tapeworm)
85
what are 2 causes of pernicious anemia
1. abs against parietal cells or abs blocking B12 complex
2. AI gastritis → loss of intrinsic factor
86
list 4 clinical presentations of B12 deficiency
1. pancytopenia
2. loss of intestinal cells → diarrhea and malabsorption
3. peripheral neuropathy
4. hypersegmented neutrophils on CBC
87
B12 deficiency causes __ neutrophils
hypersegmented
88
folate deficiency is clinically indistinguishable from
B12 deficiency
89
list 3 differences between folate deficiency and B12 deficiency
1. no neurologic symptoms in folate
2. folate is often dt dietary deficiency in US → etohism
3. folate NOT associated w. pernicious anemia
90
aplastic anemia is classified as
normocytic
91
aplastic anemia is caused by absence of \_\_
in the \_\_
RBC
bone marrow
92
what function is suppressed in aplastic anemia
stem cell fxn
93
is there splenomegaly in aplastic anemia
no!
94
what dz can aplastic anemia convert to
leukemia
95
50% of anemia is
idiopathic
96
aplastic anemia can be 2/2 to __ (5)
drug therapy
benzene
viral infxn
radiation
genetics
97
all hemolytic anemias have increased (3)
RBC destruction
erythropoiesis
iron deposition in spleen and liver
98
some hemolytic result in __ from increase in bilirubin
pigment gallstones
99
what lab will be decreased in intravascular hemolytic anemia
haptoglobin
100
what is haptoglobin and what is its fxn
protein produced by the liver
clears hgb
101
why is haptoglobin decreased in hemolytic anemias
liver can not keep up w. hemolysis/production of new RBC → haptoglobin is cleared faster than we can make it
102
what 2 lab values are elevated in intravascular hemolytic anemia
unconjugated bilirubin
LDH
103
in intravascular hemolytic anemia urine will be positive for \_\_
and \_\_, which causes
\_\_
hemosiderin
Hgb
→ causes brown urine
104
extravascular hemolysis causes __ to be more increased than in intravascular,
but **\_\_**
\_\_ are less markedly affected
unconjugated bilirubin is more elevated
haptoglobin and LDH are less markedly affected
105
extravascular hemolytic anemia is __ for hemosiderin and urine hgb
negative
106
what are 3 congenital causes of hemolytic anemia
1. G6PD deficiency
2. cytoskeleton defects
3. hemoglobinopathies
107
what are 2 acquired causes of hemolytic anemia
1. abs induced
2. mechanical
108
why does hemolytic anemia cause increased unconjugated bilirubin
unconjugated bilirubin is a breakdown product of hgb
hemolytic anemia causes destruction of hbg → increased unconjugated bilirubin
109
what is meant by intravascular hemolysis
RBC destroyed within the blood vessel
110
what is meant by extravascular hemolysis
RBC destroyed in hepatic and splenic macrophages
111
direct and indirect coombs are __ tests
antiglobulin
112
in the direct coombs test looks for abs located
on the surface of the RBC
113
the indirect coombs test looks for abs located
in the bloodstream
114
what type of coombs test is used to detect hemolytic anemia
direct
115
which type of coombs test is used to indicated a bad rxn to a transfusion
indirect
116
name 3 conditions in which coombs testing may be indicated
1. autoimmune hemolytic anemias
2. erythroblastosis fetalis
3. lupus
117
name 4 types of antibody mediated destruction of RBCs
1. incompatible RBC transfusion rxns
2. erythroblastosis fetalis
3. warm and cold autoimmune anemias
118
a positive result in coombs testing is indicated by
agglutination
119
ABO transfusion rxns are __ mediated and involve
\_\_ hemolysis
IgM
intravascular
120
isohemaglutinins are produced in what type of rxn
incompatible RBC transfusion rxns
121
erythroblastosis fetalis is also called
hemolytic dz of the newborn
122
erythroblastosis fetalis results from __ incompatibility
Rh
123
what causes the renal failure and high mortality rate in ABO transfusion reactions
hemoglobinemia
124
anti a and anti b IgM's are naturally occurring in what population
O blood type
125
non ABO transfusion reactions involve __ antibody
and __ hemolysis
IgG
extravascular
126
in non ABO transfusions, the antibody first binds to the \_\_
and then this complex is removed by the \_\_
RBC
spleen
127
non ABO rxns involve previous exposure to
non ABO antigens (ex. Rh factor)
128
what is a clinical manifestation of a non ABO transfusion rxn
jaundice
129
which Rh antigen has the most potent rxn in erythroblastosis fetalis
RhD
130
in erythroblastosis fetalis, if the mom is D (-) and the child is D (+), what is the outcome
the child is ok
131
in the second child, if the mom is D (-) and the child is D (+), what happens
IgG is present in mom, causing fetal RBC hemolysis
132
name 3 clinical presentations of erythroblastosis fetalis
1. hydrops fetalis
2. severe unconjugated hyperbilirubinemia
3. kernicterus
133
what is hydrops fetalis
severe fetal anemia
134
what is kernicterus
mental retardation caused by high levels of bilirubin in a baby's blood
135
what is the preventive tx for erythroblastis fetalis
MOC is given Rhogam (anti D antibody) during pregnancy
136
which type of coombs test is used in erythroblastosis fetalis
direct coombs test
137
in Warm Autoimmune Anemia, there is an \_\_
vs __ rxn
IgG vs self
138
why is it called “warm” autoimmune anemia
the IgG vs self rxn occurs at body temp (37C)
139
why is warm autoimmune anemia called an opsonin like rxn
opsonin tags cells for phagocytosis
in warm autoimmune anemia autoantibodies tag RBC for phagocytosis
140
what type of hemolysis is warm autoimmune anemia
extravascular
141
what are 3 clinical symptoms of warm autoimmune anemia
1. anemia
2. jaundice
3. +/- splenomegaly
142
in warm autoimmune anemia, __ coombs test will be positive
and retic count will be \_\_
direct
elevated
143
primary warm autoimmune is also called \_\_
and accounts for __ of cases
idiopathic
60%
144
name 3 causes of secondary warm autoimmune anemia
1. drugs
2. B cell neoplasm
3. lupus
145
in cold autoimmune hemolytic anemia IgM binds to RBC at __ degrees
and causes the cleavage of __ RBC
30C
C3b RBCs
146
name 2 causes of acute autoimmune hemolytic anemia
1. mycoplasma PNA
2. EBV
147
name 2 causes for chronic cold autoimmune hemolytic anemia
1. idiopathic
2. B cell neoplasms
148
in cold autoimmune hemolytic anemia, __ coombs test
is \_\_
direct
negative
149
what type of RBC morphology is associated with cold autoimmune hemolytic anemia
spherocytes
150
mechanical valves, ITP, TTP, DIC, malaria, and Babesia are all examples of what type of anemia
hemolytic
151
name 3 categories of hereditary destruction of RBC
1. defect in cytoskeleton
2. structurally abnormal hemoglobins
3. enzyme deficiency
152
hereditary spherocytosis is caused by a defect in the __ of the RBC
cytoskeleton
153
in hereditary spherocytosis, the RBC becomes less \_\_
and is trapped in the \_\_
flexible
spleen
154
which type of coombs test is used in hereditary spherocytosis, and what is the result
direct coombs
negative
155
name 2 other tests besides coombs that are associated with hereditary spherocytosis
1. increased osmotic fragility test
2. increased unconjugated bilirubin
156
name 2 clinical signs of hereditary spherocytosis
1. jaundice
2. splenomegaly
157
what is the tx for hereditary spherocytosis
splenectomy
158
what is homozygous sickle cell anemia
all normal hemoglobin is replaced with Hbs (sickled Hgb) → more severe form of sickle cell anemia
159
what is heterozygous sickle cell anemia
50% of normal hemoglobin is replaced with Hbs (sickled hgb)
160
heterozygous sickle cell anemia is considered
asymptomatic
161
sickle cell anemia disproportionately affect what patient population
African Americans
162
heterozygous sickle cell anemia (trait) is protective against what other disease
malaria
163
Hbs (sickled Hgb) is characterized as \_\_
and is made worse by what 2 factors
sticky
altitude and dehydration
164
name 6 complications of sickle cell anemia
1. microvascular obstruction → ischemia and infarction
2. emboli of necrotic bone marrow -→ acute chest syndrome
3. autosplenectomy → increased risk of infxn
4. priapism (persistent painful erection), cerebral infarcts
5. increased erythropoiesis
6. renal and pulmonary failure
165
G6PD deficiency is an X linked __ disease
recessive
166
what is the most common enzyme deficiency to cause hemolysis
G6PD deficiency
167
what 2 pt populations does G6PD deficiency-enzyme favor
middle eastern
african
168
in G6PD deficiency, the body is unable to regenerate \_\_,
which helps reduce oxidative stress
glutathione
169
name 6 oxidative factors that can exacerbate G6PD deficiency
1. antimalarial (primiquine)
2. sulfonamides
3. ASA
4. nitrofurantoin
5. viral hepatitis
6. fava beans
170
what are 2 microscopic findings associated w. G6PD deficiency
heinz bodies
bite cells
171
what do you think of when you see: red face, HA, hgb \> 60%
polycythemia
172
how does polycythemia affect RBC
increases number of RBC
173
relative polycythemia is caused by
reduced plasma volume → hemoconcentration
174
primary polycythemia is also called
polycythemia rubra vera
175
polycythemia rubra vera is a type of \_\_
that causes proliferation of \_\_
blood cancer
RBC
176
name 3 causes of secondary polycythemia
1. lung dz
2. cyanotic heart dz
3. erythropoietin producing tumors
