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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
Q

What three proteins are most commonly affected in HS>?

A

ankyrin, spectrin, or band 3, protein 4.2

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26
Q

WHat causes RBCs to become spherocytes in HS?

A

formation and loss of membrane blebs

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27
Q

How does HS cause anemia?

A

spherocytes cannot pass through splenic sinusoids and are consumed by the splenic macrophages

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28
Q

Spheroctyes have a (loss/increase) in central pallor

A

loss

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29
Q

What changes would happen to these labs in HS?RDWMCHC

A

increased RDWincreased MCHC

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30
Q

What complication of increased bilirubin presents in HS?

A

bilirubin gallstones

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31
Q

T/F: pts with HS are normally jaundiced but have normal liver function

A

true

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32
Q

Pts with HS are at an increased risk of (blank) post parvovirus B19 infection

A

aplastic crisis

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33
Q

How do you Dx HS?

A

osmotic fragility test; increased fragility

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34
Q

What is the Tx for HS?

A

splenectomy

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35
Q

What two cellular abnormalities persist on the peripheral smear after splenectomy in HS?

A

spheroctyes and howell-jolly bodies

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36
Q

Sickle cell anemia (SCA) is caused by a single amino acid mutation in the beta chain from (blank to blank)

A

glu to val

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37
Q

SCA is protective against which strain of malaria?

A

malaria falciparum

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38
Q

When does SCA become sickle cell disease?

A

mutation in both beta genes

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39
Q

what percent of Hgb is Hbs in sickle cell disease?

A

> 90%

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40
Q

What happens to HbS when it is deoxygenated?

A

polymerizes into needle like structures and causes sickling

41
Q

T/F: HbS molecules covalently bind to each other to form a tetramer upon deoxygenation

A

false; they POLYMERIZE

42
Q

An increased risk of (blank) occurs with hypoxemia, dehydration, and acidosis when you have a fucked up beta gene

A

sickling

43
Q

What type of Hb is protective against sickling?

A

HbF

44
Q

Do we see SCA in newborns?

A

nope, HbF is protective for the first few months of life

45
Q

Tx with (blank) increases levels of HbF and is used in SCA

A

hydroxyurea

46
Q

T/F: In SCA, cells continuously sickle and de-sickle

A

true

47
Q

What is the result of dynamic sickling in SCA?

A

extravascular hemolysis b/c of damaged membranes

48
Q

T/F: SCA leads to elevated bilirubin and risk for bilirubin gallstones

A

true

49
Q

T/F: SCA presents with both intra and extravascular hemolysis

A

true

50
Q

What are the lab changes associated with intravascular hemolysis in SCA?

A

decreased serum haptoglobulin

51
Q

What cell type is seen on peripheral smear as a result of intravascular hemolysis in SCA?

A

TARGET CELLS

52
Q

cErythroid hyperplasia due to SCA results in extramedullary hematopoiesis with what three characteristic findings?

A

crew cut skull xraychipmunk faciesHSM

53
Q

T/F: pts with SCA are risk of aplastic crisis

A

true, post parvovirus B19 infx

54
Q

Extensive sickling leads to complications of vaso-occlusion inluding (blank)

A

dactylitis

55
Q

Dactylitis is the vaso-occlusive event of swelling of the hands and feet from infarcts in the (bone/soft tissue)

A

bone

56
Q

(blank) is a shrunken, fibrotic spleen in response to SCA

A

autosplenectomy

57
Q

Autosplenectomy results in an increased risk to (encapsulated/naked) pathogens

A

encapsulated

58
Q

Which encapsulated pathogens pose a significant risk to pts who have autosplenectomy?

A

S. pneumoniae and H. flu

59
Q

Pts with autosplenectomy have an increased risk of osteomyelitis caused by what bug>?

A

salmonella typhi

60
Q

What abnormality can be seen on peripheral smear in patients with autosplenectomy?

A

howell jolly bodies

61
Q

(blank) is a complication of SCA that is a vaso-occlusion in pulmonary micro-circulation

A

acute chest syndrome

62
Q

Acute chest pain presents with pain, SOB, and lung (blank)

A

infiltrates

63
Q

What precipitates acute chest syndrome in pts with SCA

A

pneumonia

64
Q

What is the most common cause of death of adult SCA pts?

A

acute chest syndrome

65
Q

what is the most common cause of death of children with sCA?

A

H. flu infection

66
Q

The increased transit time of sickled RBCs through pulmonary circulation leads to increased acidemia, hypoxia, and dehydration which all promote further (blank)

A

sickling

67
Q

Renal papillary necrosis in SCA results in gross (blank and blank)

A

hematuria and proteinuria

68
Q

Sickle cell trait produces what percent of HbS?

A
69
Q

T/F: pts with sickle cell trait are normally anemic

A

false; RBCs with less than fifty percent HbS do not sickle in vivo unless in extreme circumstances

70
Q

T/F: pts with sickle cell trait have a hard time concentrating urine

A

true

71
Q

What screening test is used to test for HbS in both the trait and disease?

A

metabisulfite; will cause sickling

72
Q

What percent HbS and HbF is present in sickle cell disease?

A

90% HbS, 8%HbF

73
Q

What percent of HbS is present in sickle cell trait?

A

2%

74
Q

What is the amino acid substitution that results in HbC?

A

glu to LYSINE

75
Q

What causes the anemia in HbC?

A

extravascular hemolysis

76
Q

What is the characteristic finding on peripheral smear of HbC?

A

HbC crystals

77
Q

What is the most common protein to be altered in hereditary spherocytosis?

A

ankyrin

78
Q

Hereditary elliptocytosis is due to a defect in what two proteins?

A

spectrin tetramers or 4.1

79
Q

T/F: hereditary elliptocytosis presents with mild anemia and splenomegaly

A

true

80
Q

What disease presents this way?Strokegallstonespriapismaseptic necrosis of femoral headaplastic crisis post ParvoB19 infx

A

sickle cell anemia

81
Q

What vitamin should pts with sCA take?

A

folic acid supplementation

82
Q

Pts with G6PD def. have a susceptibilty to infections because they have an impaired (MPO/NADPH) oxidase system

A

MPO

83
Q

In what type of IHA is Raynaud’s common?

A

IgG mediated cold agglutinin

84
Q

What are the alloimmune causes of IHA?

A

Rh hemolytic disease of the newbornABO mismatchHemolytic transfusion rxn

85
Q

Drug induced IHA is (direct/indirect) coomb’s positive

A

direct coomb’s

86
Q

IgG normally does (intra/extra)vascular hemolysis

A

IgG is extraIgM is intra

87
Q

What gene is involved in PNH?

A

PIG-A gene

88
Q

T/F: PNH will present with pancyotpenia

A

true

89
Q

T/F: PNH will present with decreased haptoglobulin

A

true

90
Q

RBCs are damaged by (blank) buildup in DIC

A

fibrin

91
Q

A long distance runner may suffer what type of hemolysis?

A

traumatic

92
Q

Snake venoms, malaria, babesia, burns, and clostridium perfringens cause what type of anemia?

A

non-immune hemolytic anemia

93
Q

Describe the following lab values in regard to hemolytic disease of the newborn:DATTotal and indirect biliLDHHCT and Hgb

A
  1. pos DAT2. increased total and indirect bili3. increased LDH4. INITIALLY HCT and Hgb WNL
94
Q

At what level of BUN does uremia present with anemia?

A

when BUN is twice normal

95
Q

What type of cells are seen on peripheral smear in uremia?

A

Burr cells (echinocytes) spiny projections

96
Q

T/F: traumatic hemolysis can form burr cells

A

true

97
Q

Describe the size and color of uremic anemia

A

norchromic normocytic with MILD anisocytosis

98
Q

Anemia with neoplasia will be normocrhomic normocytic anemia unless…(three things)

A
  1. blood loss2. hemorrhage3. myelophthistic process
99
Q

What is the most common cause of nonmegaloblastic macrocytic anemia?

A

alcoholism

PATHOMA (11 decks)

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