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PATHOMA > Ch. 5 - Normocytic with Extravascular Hemolysis > Flashcards

Ch. 5 - Normocytic with Extravascular Hemolysis Flashcards

1
Q

What lab value helps you determine if a normocytic anemia is from over destruction or underproduction?

A

RETC

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2
Q

WHat is a normal RETC

A

1-2%

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3
Q

A properly functioning bone marrow responds to anemia by increasing the RETC to (blank)

A

> 3%

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4
Q

T/F: RETC is falsely elevated in anemia

A

true

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5
Q

How is RETC falsely elevated in anemia?

A

percentage of total RBCs, so if the total count of RBCs decreases, then the RETC looks higher than it should be

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6
Q

How do you calculate the corrected RETC?

A

(Hct/45) x RETC

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7
Q

A corrected RETC >3% tells you what?

A

good marrow response and suggests PERIPHERAL DESTRUCTION

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8
Q

A corrected RETC

A

bad marrow response and suggests UNDERPRODUCTION

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9
Q

T/F: intravascular and extravascular hemolysis both present as an anemia with a good marrow response

A

true

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10
Q

What are the following components of RBCs broken down into?1. globin2. heme3. protoporphyrin

A
  1. amino acids2. iron and protoporphyrin; iron is recycled3. unconjugated bilirubin, bound ot serum albumin and delivered to the liver for conjugation and excretion into the bile
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11
Q

Peripheral RBC destruction will result in (splenomegaly/HSM)

A

splenomegaly only, but jaundice!

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12
Q

What painful dietary complication are pts at risk of developing with increased levels of bilirubin?

A

bilirubin gallstones

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13
Q

T/F: peripheral blood cell destruction results in marrow hyperplasia

A

true

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14
Q

Is this intravascular or extravascular?HemoglobinemiaHemoglobinuriaHemosiderinuria

A

INTRAvascular

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15
Q

Why do pts with intravascular hemolysis present with hemosiderinuria? When does this develop?

A

renal tubular cells pick up some of the hemoglobin that is filtered into the urine and break it down into iron, which accumulates as hemosiderin; tubular cells are eventually shed resulting in hemosiderinura.

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16
Q

Is serum haptoglobin increased or decreased in intravascular hemolysis?

A

decreased; hapto is a scavenger molecule that will try to take up the free Hgb in the blood

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17
Q

What range of increased LDH would we see for extravascular hemolysis?

A

500-700

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18
Q

What is the first organ to sense hypoxia and what hormone becomes elevated in extra. hemolysis?

A

kidney; increased EPO

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19
Q

In a chronic extravascular hemolysis, what characteristics of the anemia are added that complicate the DDx?

A

increased folate requirements and iron deficiency which could make you think it was a folate def or simple iron def. anemia

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20
Q

What are the five things you think of when you see a macrocytic anemia?

A

Folate def.Vit. B12 def.AlcoholismMDSLiver disease

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21
Q

What are the two things that increase the RETC count>?

A

bleeding or hemolysis

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22
Q

If spherocytes are present on the peripheral smear, what are the only two things on your DDx?

A
  1. autoimmune hemolytic anemia2. Hereditary spherocytosis
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23
Q

What other clues can we get from a CBC that lets us know that the cause of the anemia is from an underproduction in the bone marrow

A

you will likely see that other cell lines are low too

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24
Q

What is the molecular cause of Hereditary spherocytosis? (HS)

A

RBC cytoskeleton-membrane tethering proteins

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25
What three proteins are most commonly affected in HS>?
ankyrin, spectrin, or band 3, protein 4.2
26
WHat causes RBCs to become spherocytes in HS?
formation and loss of membrane blebs
27
How does HS cause anemia?
spherocytes cannot pass through splenic sinusoids and are consumed by the splenic macrophages
28
Spheroctyes have a (loss/increase) in central pallor
loss
29
What changes would happen to these labs in HS?RDWMCHC
increased RDWincreased MCHC
30
What complication of increased bilirubin presents in HS?
bilirubin gallstones
31
T/F: pts with HS are normally jaundiced but have normal liver function
true
32
Pts with HS are at an increased risk of (blank) post parvovirus B19 infection
aplastic crisis
33
How do you Dx HS?
osmotic fragility test; increased fragility
34
What is the Tx for HS?
splenectomy
35
What two cellular abnormalities persist on the peripheral smear after splenectomy in HS?
spheroctyes and howell-jolly bodies
36
Sickle cell anemia (SCA) is caused by a single amino acid mutation in the beta chain from (blank to blank)
glu to val
37
SCA is protective against which strain of malaria?
malaria falciparum
38
When does SCA become sickle cell disease?
mutation in both beta genes
39
what percent of Hgb is Hbs in sickle cell disease?
>90%
40
What happens to HbS when it is deoxygenated?
polymerizes into needle like structures and causes sickling
41
T/F: HbS molecules covalently bind to each other to form a tetramer upon deoxygenation
false; they POLYMERIZE
42
An increased risk of (blank) occurs with hypoxemia, dehydration, and acidosis when you have a fucked up beta gene
sickling
43
What type of Hb is protective against sickling?
HbF
44
Do we see SCA in newborns?
nope, HbF is protective for the first few months of life
45
Tx with (blank) increases levels of HbF and is used in SCA
hydroxyurea
46
T/F: In SCA, cells continuously sickle and de-sickle
true
47
What is the result of dynamic sickling in SCA?
extravascular hemolysis b/c of damaged membranes
48
T/F: SCA leads to elevated bilirubin and risk for bilirubin gallstones
true
49
T/F: SCA presents with both intra and extravascular hemolysis
true
50
What are the lab changes associated with intravascular hemolysis in SCA?
decreased serum haptoglobulin
51
What cell type is seen on peripheral smear as a result of intravascular hemolysis in SCA?
TARGET CELLS
52
cErythroid hyperplasia due to SCA results in extramedullary hematopoiesis with what three characteristic findings?
crew cut skull xraychipmunk faciesHSM
53
T/F: pts with SCA are risk of aplastic crisis
true, post parvovirus B19 infx
54
Extensive sickling leads to complications of vaso-occlusion inluding (blank)
dactylitis
55
Dactylitis is the vaso-occlusive event of swelling of the hands and feet from infarcts in the (bone/soft tissue)
bone
56
(blank) is a shrunken, fibrotic spleen in response to SCA
autosplenectomy
57
Autosplenectomy results in an increased risk to (encapsulated/naked) pathogens
encapsulated
58
Which encapsulated pathogens pose a significant risk to pts who have autosplenectomy?
S. pneumoniae and H. flu
59
Pts with autosplenectomy have an increased risk of osteomyelitis caused by what bug>?
salmonella typhi
60
What abnormality can be seen on peripheral smear in patients with autosplenectomy?
howell jolly bodies
61
(blank) is a complication of SCA that is a vaso-occlusion in pulmonary micro-circulation
acute chest syndrome
62
Acute chest pain presents with pain, SOB, and lung (blank)
infiltrates
63
What precipitates acute chest syndrome in pts with SCA
pneumonia
64
What is the most common cause of death of adult SCA pts?
acute chest syndrome
65
what is the most common cause of death of children with sCA?
H. flu infection
66
The increased transit time of sickled RBCs through pulmonary circulation leads to increased acidemia, hypoxia, and dehydration which all promote further (blank)
sickling
67
Renal papillary necrosis in SCA results in gross (blank and blank)
hematuria and proteinuria
68
Sickle cell trait produces what percent of HbS?
69
T/F: pts with sickle cell trait are normally anemic
false; RBCs with less than fifty percent HbS do not sickle in vivo unless in extreme circumstances
70
T/F: pts with sickle cell trait have a hard time concentrating urine
true
71
What screening test is used to test for HbS in both the trait and disease?
metabisulfite; will cause sickling
72
What percent HbS and HbF is present in sickle cell disease?
90% HbS, 8%HbF
73
What percent of HbS is present in sickle cell trait?
2%
74
What is the amino acid substitution that results in HbC?
glu to LYSINE
75
What causes the anemia in HbC?
extravascular hemolysis
76
What is the characteristic finding on peripheral smear of HbC?
HbC crystals
77
What is the most common protein to be altered in hereditary spherocytosis?
ankyrin
78
Hereditary elliptocytosis is due to a defect in what two proteins?
spectrin tetramers or 4.1
79
T/F: hereditary elliptocytosis presents with mild anemia and splenomegaly
true
80
What disease presents this way?Strokegallstonespriapismaseptic necrosis of femoral headaplastic crisis post ParvoB19 infx
sickle cell anemia
81
What vitamin should pts with sCA take?
folic acid supplementation
82
Pts with G6PD def. have a susceptibilty to infections because they have an impaired (MPO/NADPH) oxidase system
MPO
83
In what type of IHA is Raynaud's common?
IgG mediated cold agglutinin
84
What are the alloimmune causes of IHA?
Rh hemolytic disease of the newbornABO mismatchHemolytic transfusion rxn
85
Drug induced IHA is (direct/indirect) coomb's positive
direct coomb's
86
IgG normally does (intra/extra)vascular hemolysis
IgG is extraIgM is intra
87
What gene is involved in PNH?
PIG-A gene
88
T/F: PNH will present with pancyotpenia
true
89
T/F: PNH will present with decreased haptoglobulin
true
90
RBCs are damaged by (blank) buildup in DIC
fibrin
91
A long distance runner may suffer what type of hemolysis?
traumatic
92
Snake venoms, malaria, babesia, burns, and clostridium perfringens cause what type of anemia?
non-immune hemolytic anemia
93
Describe the following lab values in regard to hemolytic disease of the newborn:DATTotal and indirect biliLDHHCT and Hgb
1. pos DAT2. increased total and indirect bili3. increased LDH4. INITIALLY HCT and Hgb WNL
94
At what level of BUN does uremia present with anemia?
when BUN is twice normal
95
What type of cells are seen on peripheral smear in uremia?
Burr cells (echinocytes) spiny projections
96
T/F: traumatic hemolysis can form burr cells
true
97
Describe the size and color of uremic anemia
norchromic normocytic with MILD anisocytosis
98
Anemia with neoplasia will be normocrhomic normocytic anemia unless...(three things)
1. blood loss2. hemorrhage3. myelophthistic process
99
What is the most common cause of nonmegaloblastic macrocytic anemia?
alcoholism

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