Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Hema 3 > 3.2 > Flashcards

3.2 Flashcards

1
Q

serves as a barrier

A

Lipid Bilayer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

the ability to repeatedly bend, stretch, distort and return to normal discoid shape.

A

Deformability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Elasticity- interactions between Spectrin and junctional
complexes.

A

Pliancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

concentration of Hemoglobin and
maintenance of proper cell volume.

A

Cytoplasmic Viscosity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Perpendicular to cytoskeleton, prevents loss of membrane= decreased surface area-to-volume ratio of RBC (Names)

A

Vertical (Structural Integrity)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Prevents membrane from fragmenting in response to mechanical stress. (Names)

A

Horizontal (Mechanical stability)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the hereditary diseases that are defective in their membrane structures

A

H. Spherocytosis
H. Elliptocytosis
H. Ovalocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the hereditary diseases that are defective in their membrane transport proteins

A

H. hydrocytosis
H. xerocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Acquired RBC membrane abnormalities

A

A. stomattocytosis
Spur cell
Paroxysmal nocturnal hemoglobinuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

In H. spherocytosis, what are the mutated gene

A

B- Spectrin
A-Spectrin
Band 3
Ankyrin
Protein 4.2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What kind of trait is hereditary spherocytosis

A

75% Autosomal Dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Defects/ Mutations in proteins that disrupt the vertical interactions between
transmembrane proteins and cytoskeleton.

A

Hereditary Spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

RBCs lose unsupported lipid membranes and acquire a decreased surface area-to volume
ratio

A

SPHEROCYTES

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What happens if the RBC membranes have abnormal permeability to cations?

A

Increase in Viscosity and cellular dehydration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

No deformability of a normal discoid RBC.

A

HEREDITARY SPHEROCYTOSIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Asymptomatic, RBC loss is compensated.

A

MILD HS (20-30%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Normal Hgb, Slight Increase in Bilirubin, Increased reticulocytes, and
presence of few spherocytes in Peripheral Blood Smear

A

MILD HS (20-30%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Usually seen in pregnancy, Infectious Mononucleosis, or in aging

A

MILD HS (20-30%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Hgb >8g/dL, Serum Bilirubin >2mg/dL, Reticulocyte count >8%

A

MODERATE HS (60%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Spherocytes in PBS.

A

MODERATE HS (60%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Hgb 6-8g/dL, Serum Bilirubin 2-3mg/dL, Reticulocyte count >10%

A

MODERATE to SEVERE HS (5-10%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Hgb <6g/dL, Serum Bilirubin >3mg/dL, Reticulocyte count >10%

A

SEVERE HS (3-5%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Require regular transfusions

A

SEVERE HS (3-5%):

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Hallmark of HS

A

Spherocytes:

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

small, round, dense RBCs
filled with Hgb, no central pallor.

A

Microspherocytes:

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

RBC Morphology:

Mutated gene in B-spectrin

A

Acanthocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

RBC Morphology:

Mutated gene in Band 3

A

Pincered or mushroom shaped cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

RBC Morphology:

Mutated gene in EPB4.2

A

Ovalostomatocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

True or False

Spherocytes are specific for hereditary spherocytosis

A

FALSE

Spherocytes are NOT specific for HS.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q
  • MCHC: > 36mg/dL (for moderate to severe)
  • MCV: WIR to slightly below
A

HEREDITARY SPHEROCYTOSIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Lab Findings in H. Spherocytosis:

Serum Haptoglobin

Increase or Decrease?

A

Decrease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Lab Findings in H. Spherocytosis:

Serum Indirect Bilirubin

Increase or Decrease?

A

Increase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Lab Findings in H. Spherocytosis:

Serum Lactate Dehydrogenase

Increase or Decrease?

A

Increase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

In the Bone Marrow Exam of hereditary spherocytosis, what will you see? Is it required for diagnosis?

A

Erythroid Hyperplasia

No, not required for diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Demonstrates Increased RBC Fragility on RBCs
with decreased surface area-to-volume ratios.

A

OSMOTIC FRAGILITY TEST

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

In OSMOTIC FRAGILITY TEST, RBCs are subjected to increasing ________
solutions.

A

hypotonic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Shift to the ____, Increased Osmotic Fragility

A

LEFT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Shift to the _____, Decreased Osmotic Fragility

A

Right

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

In osmotic fragility test

Time-consuming, Needs Fresh _______
sample collected without ______, accurately
prepared _____ solutions.

A

Heparinized; Trauma; NaCl

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Confirmatory test for HS. More sensitive alternative to EOFT

A

EOSIN-5’-MALEIMIDE BINDING TEST

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Measures Mean Fluorescence Intensity.

A

EOSIN-5’-MALEIMIDE BINDING TEST

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

rare associated with viral syndromes i.e. Infectious Mononucleosis.
Spleen enlarges, sluggish blood flow and destroys RBCs.

A

HEMOLYTIC CRISIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Marked decrease in reticulocytes. Associated with Parvovirus B19 infection,
destroys progenitor cells in BM and suppresses erythropoiesis.

A

APLASTIC CRISIS-

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Increased Folate utilization. Particularly acute during pregnancy.

A

MEGALOBLASTIC CRISIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Treatment for H. Spherocytosis

A
  • Regular Transfusion
  • Splenectomy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Differential Diagnosis in H. spherocytosis

A
  • HS: Negative DAT (Direct Antiglobulin Test)
  • EMA Binding Test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Treatment for H. Elliptocytosis

A
  • Regular Transfusion
  • Splenectomy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

what trait is the hereditary elliptocytosis?

A

Autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Mutated gene in

a- Spectrin,
B-Spectrin,
Protein 4.1

What disease?

A

hereditary elliptocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Weaken the Mechanical Stability of the
membrane

A

hereditary elliptocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

More common in Africa and Mediterranean
regions

A

hereditary elliptocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Defects/ Mutations in proteins that disrupt the horizontal/ lateral interactions in
the protein cytoskeleton.

A

hereditary elliptocytosis

53
Q

Normal, discoid RBCs become elliptical from repeated exposure to shear stress in
the peripheral circulation

A

ELLIPTOCYTES

54
Q

ELLIPTOCYTOSIS IN PATIENTS WITH LEACH PHENOTYPE lacks what?

A

Lacks Gerbich Antigens and GPC

55
Q

Mutations in Glycoprotein C what are the defect?

A

GPC and protein 4.1 interactions

56
Q

Severe form of Hereditary Elliptocytosis

A

HEREDITARY PYROPOIKILOCYTOSIS

57
Q

Decreased Thermal Stability. (Marked RBC fragmentation on heating).

A

HEREDITARY PYROPOIKILOCYTOSIS

58
Q

Extreme Poikilocytosis with fragmentation, microspherocytosis and
elliptocytosis (similar to patients with thermal burns).

A

HEREDITARY PYROPOIKILOCYTOSIS

59
Q

MCV is very low (50 to 65fL)
- Lower fluorescence in EMA binding test compared to HS.

A

HEREDITARY PYROPOIKILOCYTOSIS

60
Q

Diseases that are less than 1/3 of the RBCs are elliptocytes (TIMPMM)

A

(IDA,Thalassemia,megaloblastic anemia, MDS,Primary Myelofibrosis

61
Q

Cholelithiasis can develop.

A

HEREDITARY ELLIPTOCYTOSIS and spherocytosis

62
Q

Southeast Asian Ovalocytosis (SAO)

A

HEREDITARY OVALOCYTOSIS

63
Q

WHat trait is HEREDITARY OVALOCYTOSIS?

A

Mainly Autosomal Dominant

64
Q

WHat mutated gene has HEREDITARY OVALOCYTOSIS

A

Band 3

65
Q

True or False

HEREDITARY OVALOCYTOSIS decreases in membrane rigidity?

A

False; Increases

66
Q

More common in the malaria belt of
Southeast Asia

A

HEREDITARY OVALOCYTOSIS

67
Q

Results from only one mutation, deletion of 27 base pairs in the gene SLC4A1 which
codes for Band 3.

A

HEREDITARY OVALOCYTOSIS

68
Q

In HEREDITARY OVALOCYTOSIS, Increase membrane rigidity due to a tighter binding of Band 3 to _____.

A

Ankyrin

69
Q

True or False

Hereditary Ovalocytes RBC membranes have decreased elasticity.

A

True

70
Q

Overhydrated hereditary stomatocytosis (OHS)

A

HEREDITARY HYDROCYTOSIS

71
Q

What inheritance trait is HEREDITARY HYDROCYTOSIS

A

Autosomal dominant trait

72
Q

What is the Deficient Protein/ Mutated Gene in HEREDITARY HYDROCYTOSIS

A

RhAg

73
Q

What is the secondary Deficient Protein/ Mutated Gene in HEREDITARY HYDROCYTOSIS

A

Stomatin

74
Q

RBC membrane is excessively permeable to Sodium and Potassium at 37oC.

A

HEREDITARY HYDROCYTOSIS

75
Q

Influx of Na+ into the cell, increase loss of K+ . More water enters the cell causing it
to swell

A

Hereditary Hydrocytosis Stomatocytosis

76
Q

Dehydrated hereditary stomatocytosis (DHS)

A

HEREDITARY XEROCYTOSIS

77
Q

WHat trait is HEREDITARY XEROCYTOSIS

A

Autosomal dominant

78
Q

Deficient Protein/ Mutated Gene: PIEZO 1
Protein, KCNN4

A

HEREDITARY XEROCYTOSIS

79
Q

Most common form of Stomatocytosis

A

HEREDITARY XEROCYTOSIS

80
Q

In HEREDITARY XEROCYTOSIS, RBC membrane is excessively permeable to ________.

A

Potassium

81
Q

Increase loss of K+ with no increase in Sodium. Reduced Intracellular cation
concentration, Water is lost from the cell.

A

Hereditary Xeroxytosis

82
Q

What disease has the clinical findings of Fetal loss, hydrops fetalis and neonatal hepatitis.

A

Hereditary Xeroxytosis

83
Q

Rare Autosomal Dominant disorder, mutation in ABCB6 gene.

A

FAMILIAL PSEUDOHYPERKALEMIA

84
Q

In FAMILIAL PSEUDOHYPERKALEMIA, Excessive loss of RBC K+ at room temperature in ____but not at body temp in ____.

A

Vitro, vivo

85
Q

Cold induced leakage of Na+ and K+ from the RBCs.

A

CRYOHYDROCYTOSIS

86
Q

Increased cation permeability, cell swelling, and hemolysis when stored at ____for 24
to 48 hours.

A

4

87
Q

Rh membrane proteins is absent (Rh-Null) or decreased (Rh-mod).

A

Rh deficiency syndrome

88
Q

Rare autosomal recessive disorder. Mutation in MTP needed to transfer and assemble lipids

A

Abetalipoproteinemia (ABL)

89
Q

In ABL, RBC membrane acquires increased S___________ which decreases fluidity of membrane and results in shape change.

A

Sphingomyelin

90
Q

What trait is MLS?

A

X-linked disorder

91
Q

Mutations in KX Gene, precursor for Kell Blood Group Antigens

A

McLeod Syndrome (MLS)

92
Q

In MLS, Reduced expression of Kell Ag, reduced RBC deformability, ________RBC survival

A

shortened

93
Q

Rare Autosomal recessive, mutaions in Chorein which leads to abnormal membrane structure

A

Chorea Acanthocytosis (ChoAc)

94
Q

Excess Free Cholesterol accumulates in the RBC membrane. The spleen
remodels the RBC giving long, rigid projections

A

ACANTHOCYTES

95
Q
  • Drying Artifact on Peripheral Blood Smear
  • Acute Alcoholism
  • Malignancies
  • Cardiovascular diseases
A

Acquired stomatocytosis

96
Q

Acquired- mutations occur after birth

A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

97
Q

Intravascular hemolytic anemia from mutations in PIGA Gene encodes GPI, anchor protein.

A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

98
Q

In PNH, No GPI, No complement regulatory Proteins CD__and CD__.

A

CD55 and CD59.

99
Q

Phenotypic mosaicism􀍟􀑁

A

PNH

100
Q

No CD55 and CD59 complement attack the RBC, causing i____________ lysis.

A

intravascular

101
Q

Causes Smooth Muscle Dystonia (Esophageal spasma,dysphagia, erectile dysfunction)

A

PNH

102
Q

Causes thrombosis (i.e. hepatic- Budd-Chiarri syndrome)

A

PNH

103
Q
  • Chronic renal disease
A

PNH

104
Q

Subcategories of PNH:

30-50% of circulating Neutrohpils are GPI deficient

A

Classic PNH

105
Q

Subcategories of PNH:

<20-30% of Neutrophils are GPI deficient

A

Hypoplastic PNH

106
Q

Subcategories of PNH:

GPI deficient neutrophils is less than 1%V

A

Subclinical PNH

107
Q

Hemoglobinuria at Night, Hemosidenuria

A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

108
Q

What are the test in PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (3)

A

Sugar Water Test (Sucrose Hemolysis Test) and HAM Test (Acidified Serum Lysis Test), and LapScore

109
Q

Treatment:

binds complement C5, inhibits formation of membrane attack unit.

For what disease?

A

Eculizumab for PNH

110
Q

Treatment:

Hematopoietic Stem Cell Transplant

A

PNH

111
Q

In PNH, __________ __________ vaccine before drug administration. (bacteria)

A

Neiserria meningitidis

112
Q

The most common RBC enzyme defect and prevalence of 5% of the global population.

The highest prevalence where malaria is endemic

A

G6PD def

113
Q

WHat pattern of inheritance is G6PD def

A

x-linked

114
Q

G6PD provides the only means to generate _____ needed to protect the cells from
oxidative ______.

A

NADPH; stress

115
Q

Oxidative agents/stressors in G6PD

A

Infection, Drugs, Fava Beans

116
Q

In G6PD def:

O___________ converts hemoglobin to methemoglobin, d _________the Hgb solubility of the cell, and precipitates as H______ B______ which adhere to the inner RBC membrane and are removed by hemolysis.

A

Oxidation; decreases; Heinz Bodies

117
Q

rare, severe hemolytic episode following ingestion of fava beans.

A

Favism

118
Q

Jaundice appears 2-3 days after birth may lead
to kernicterus.

A

Neonatal hyperbilirubinemia

119
Q

diagnosed as neonatal hyperbilirubinemia at birth but persists to adulthood.

No Hemoglobinuria, suggests extravascular hemolysis

A

Chronic hereditary nonspherocytic hemolytic anemia

120
Q

Gold standard for G6PD def

A

Quantitative Spectrophotometric Assays

121
Q

Rapid Screening for G6PD

A

Qualitative/ Spot Tests

122
Q

Most common form of hereditary non-spherocytic hemolytic anemia

A

Pk def

123
Q

What inheritance pattern in PK def?

A

Autosomal recessive

124
Q

In PK def, Mutation on ____gene that codes for PK in RBCs

A

PKLR

125
Q

If there is NO Pyruvate Kinase, NO ATP. RBCs are dehydrated, what rbc morphology will form ?

A

Echinocytes (Burr Cells)

126
Q

Glycolytic Pathway is blocked, increasing 2,3- BPG

Will Shift to ______

A

Right

127
Q

3rd most common RBC enzyme deficiency.

A

Pyrimidine 5’ nucleotidase type 1

128
Q

In P5’NT-1, B________ s_______ in the cell leading to premature hemolysis.

A

Basophilic Stippling

Hema 3 (2 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions