Ch. 5 - Normocytic with Extravascular Hemolysis Flashcards Preview

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Flashcards in Ch. 5 - Normocytic with Extravascular Hemolysis Deck (99):
1

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

RETC

2

WHat is a normal RETC

1-2%

3

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

>3%

4

T/F: RETC is falsely elevated in anemia

true

5

How is RETC falsely elevated in anemia?

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

6

How do you calculate the corrected RETC?

(Hct/45) x RETC

7

A corrected RETC >3% tells you what?

good marrow response and suggests PERIPHERAL DESTRUCTION

8

A corrected RETC

bad marrow response and suggests UNDERPRODUCTION

9

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

true

10

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

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

11

Peripheral RBC destruction will result in (splenomegaly/HSM)

splenomegaly only, but jaundice!

12

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

bilirubin gallstones

13

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

true

14

Is this intravascular or extravascular?HemoglobinemiaHemoglobinuriaHemosiderinuria

INTRAvascular

15

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

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.

16

Is serum haptoglobin increased or decreased in intravascular hemolysis?

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

17

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

500-700

18

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

kidney; increased EPO

19

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

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

20

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

Folate def.Vit. B12 def.AlcoholismMDSLiver disease

21

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

bleeding or hemolysis

22

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

1. autoimmune hemolytic anemia2. Hereditary spherocytosis

23

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

you will likely see that other cell lines are low too

24

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

RBC cytoskeleton-membrane tethering proteins

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