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Clinical Hematology > Test 4 review sheet > Flashcards

Test 4 review sheet Flashcards

1
Q

hereditary spherocytosis is highest in people of ?

A

Northern European ancestry

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2
Q

Hereditary spherocytosis

A

membrane protein deficiencies that result in an unsupported red cell membrane

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3
Q

spherocytes in hereditary spherocytosis

A

rigid and not as deformable as the normal biconcave red cell

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4
Q

What does the spleen do to correct the spherocytes in hereditary spherocytosis?

A

sequesters these abnormal red cells, where they become further damaged and are selectively removed by macrophages

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5
Q

direct antiglobulin test

A

detects antibodies present on the red blood cell (RBC) membrane

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6
Q

If the antiglobulin test is negative?

A

antibodies won’t be present and thus be a sign of hereditary spherocytosis

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7
Q

Tests for hereditary spherocytosis

A

Direct antiglobulin test
Osmosis fragility
Red cell morphology
reticulocyte count

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8
Q

Results of test if hereditary spherocytosis is present

A

Direct antiglobulin test: negative
Osmosis fragility: increased
Red cell morphology: spherocytes present
reticulocyte count: increased

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9
Q

aplastic crisis.

A

a temporary shutdown of red cell production due to a drop in hemoglobin from a viral infection

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10
Q

What is the difference between immune hemolytic anemias (with spherocytes) and hereditary spherocytosis

A

Direct antiglobulin test:
Positive for IHA and negative for HS

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11
Q

What happens to MCHC in hereditary spherocytosis?

A

increased, probably for cellular dehydration from cells with low levels of water and potassium

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12
Q

What temperatures can affect osmotic fragility?

A

incubaton at 37 degrees C for 24 hours increases fragility

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13
Q

Why does temperature affect osmotic fragility?

A

red cells become metabolically deprived and tend to lose membrane surface because of their relative membrane instability

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14
Q

What happens after a splenectomy?

A

anemia is usually corrected and reticulocyte counts return to high-normal

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15
Q

Consequences of a splenectomy?

A

appearance of Howell-Jolly bodies
siderocytes
target cells in red cells

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16
Q

What won’t a splenectomy change?

A

Spherocytes in peripheral blood

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17
Q

Hereditary elliptocytosis treatments

A

Splenectomy, though if severe a transfusion before the procedure

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18
Q

Aspect of Hereditary elliptocytosis

A

RBCs appear more elliptical

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19
Q

Where can burr cells (Echinocytes) be observed

A

Patients with kidney problems and newborns

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20
Q

Acanthocytes can be seen where?

A

abetalipoproteinemia
liver disease
McLeod blood groups

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21
Q

Which group has G6PD A deficiency?

A

African American males

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22
Q

G6PD A gene is linked to?

A

The gene is X linked, so that female subjects are carriers and generally do not have clinical problems

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23
Q

G6PD A are clinically normal unless

A

exposed to an oxidant drug, This can result in acute hemolysis

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24
Q

Bite cells and polychromasia can be seen through?

A

Wright stain

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25
Q

Heinz bodies can’t be seen under a?

A

Wright Stain

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26
Q

What is higher in reticulocytes than older RBCs?

A

G6PD, Because reticulocytes are increased after a hemolytic episode, their presence can give a normal result in the screening test for G6PD

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27
Q

Pyruvate kinas

A

the most common enzyme deficiency in the glycolytic pathway

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28
Q

the most common red cell morphologic findings in PK deficiency

A

Polychromasia and echinocytes

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29
Q

diagnosis of PK deficiency

A

performing an assay for the enzyme

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30
Q

Red cells in paroxysmal nocturnal hemoglobinuria are abnormally sensitive

A

to complement, which is activated on their surface and results in premature destruction of the red cells

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31
Q

Hemosiderin

A

present and a useful diagnostic tool in paroxysmal nocturnal hemoglobinuria

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32
Q

What occurs during paroxysmal nocturnal hemoglobinuria

A

CD55 decrease
Hemolysis when blood is acidic
Platelets and WBCs are decreased

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33
Q

What tool is good for diagnosis paroxysmal nocturnal hemoglobinuria?

A

Flow cytometer, though has been replaced by sucrose hemolysis and Ham

34
Q

What can hereditary spherocytosis be inherited as?

A

an autosomal dominant trait, although autosomal recessive forms exist

35
Q

G6PD gene

A

has the greatest degree of variability in the human genome; this results in multiple clinical variants
Mainly found on the X Chromosome

36
Q

G6PD B vs G6PD A

A

G6PD A contains one mutation so that its electrophoretic pattern is different, but it is a benign change

37
Q

G6PD is needed for what?

A

regenerate reduced glutathione in the red cell

38
Q

Reduced glutathione

A

essential to generate nicotinamide adenine dinucleotide phosphate, which is necessary to prevent oxidative damage to the red cell

39
Q

Paroxysmal nocturnal hemoglobinuria is what kind of disease?

A

An acquired disease that can be shown to be clonal in nature

40
Q

What are surface proteins of paroxysmal nocturnal hemoglobinuria linked to

A

GPI, which link it to the membrane

41
Q

PIGA gene

A

Located on the X chromosome, , which is mutated in paroxysmal nocturnal hemoglobinuria

42
Q

examples of microangiopathic hemolytic anemia (MAHA)s

A

Thrombotic thrombocytopenia purpura
hemolytic uremic syndrome
DIC

43
Q

causes immune thrombocytopenia purpura

A

An antibody against platelets

44
Q

MAHA is characterized by what?

A

red cell fragmentation; schistocytes are thus seen in the blood

45
Q

What happens when RBCs are forced into the fibrin?

A

they are fragmented, as pass through fibrin deposits that have been abnormally formed inside the lumen of blood vessels

46
Q

How else might RBCs fragment?

A

turbulence of red cells as they pass over damaged endothelial cells that line the blood vessels

47
Q

What can result in MAHA?

A

infections (sepsis)
obstetrics complications
cancer

48
Q

Exercise-induced hemoglobinuria occurs when?

A

individuals after repeated forceful effect of the feet on hard surfaces. Hence the name march hemoglobinuria

49
Q

How many people are killed by malaria?

A

more than 1 million people per year

50
Q

Plasmodium falciparum

A

Most serious form of malaria, has banana shaped gametocytes

51
Q

How doe clinicians diagnose malaria

A

Blood films

52
Q

What can 3rd degree burn patients develope?

A

severe acute hemolytic anemia

53
Q

Cell morphology of severe acute hemolytic anemia

A

globular fragmentation
budding
microspherocytes

54
Q

When does acute hemolytic anemia begin to subside

A

24 hours after the injury before they are removed from circulation.

55
Q

What is prominent in hemolytic uremic syndrome?

A

Renal failure

56
Q

What can decrease malaria infection of RBCs

A

Heterozygosity (Genetic variability) for Hb S
RBCs lacking the Duffy phenotype
Rh-negative genotype
hereditary spherocytosis

57
Q

P. falciparum can infect what type of RBCs

A

RBCs in all stages of development

58
Q

What does thin and thick blood films

A

Thick: concentrate the parasites and are ideal for initial screening
Thin: determination of the percent of parasitemia, speciation, and staging

59
Q

patients with thrombotic thrombocytopenic purpura typically have?

A

A severe deficiency of von Willebrand factor (VWF)–cleaving protease

60
Q

Rh hemolytic disease of the newborn are typically found in?

A

Mothers with no prenatal care, however mother’s antibody titer does not predict the severity of hemolytic disease

61
Q

IgG autoantibodies causes?

A

Red cell destruction in warm-reactive autoimmune hemolytic anemia

62
Q

IgG-sensitized red blood cells are resolved by?

A

The macrophages in the spleen which remove them from circulation

63
Q

direct antiglobulin test

A

tests that distinguishes warm autoimmune hemolytic anemia from hereditary spherocytosis

64
Q

direct antiglobulin test results

A

Negative: hereditary spherocytosis
Positive: warm autoimmune hemolytic anemia

65
Q

Cold-reactive autoimmune hemolytic anemia

A

secondary after infections with organisms such as M. pneumoniae and Epstein Barr Virus

66
Q

What happens when blood agglutination is on a blood film

A

blood should be warmed to 37C for 15 minutes and the automated analysis of the specimen repeated while the blood is warm

67
Q

What antibody is not associated with excess red cell destruction?

A

IgE antibodies

68
Q

cause RBC destruction

A

. Drugs
IgM autoantibodies
IgG alloantibodies

69
Q

largest number of deaths

A

ABO blood type errors from patient misidentification

70
Q

What can cause an immediate infusion reaction?

A

ABO incompatibility, is most often the result of human error

71
Q

Erythroblastosis results in?

A

ABO or anti-D, occurs as a result of a maternal-fetal incompatibility

72
Q

A delayed hemolytic transfusion reaction occurs?

A

as a result of undetected alloantibodies, where the level of antibody was below the level of sensitivity at the time of transfusion

73
Q

When only complement is present on the membrane

A

anti-C3b/C3d, a cold agglutinin is most likely present, thus agglutination would be seen at room temp.

74
Q

In infants whose mothers make an IgG anti-A or anti-B at a low level isn’t really bad because

A

Most ABO antibodies are IgM and do not cross the placenta because of their large size

75
Q

Evans syndrome

A

When thrombocytopenia and a warm-reactive autoimmune hemolytic anemia are found together, usually in children

76
Q

Wiskott-Aldrich syndrome

A

X-linked and is caused by a mutation in a gene that encodes a protein called WASp

77
Q

Wiskott-Aldrich syndrome results

A

low levels or absence of WASp, and affected individuals have immunodeficiency, eczema, and thrombocytopenia

78
Q

Hereditary spherocytosis can arise from what?

A

defects in proteins that provide vertical support for the membrane

79
Q

Hereditary elliptocytosis can result from

A

defects in cytoskeletal proteins that provide horizontal
support for the membrane

80
Q

Donath-Landsteiner antibody can be found

A

on paroxysmal cold hemoglobinuria

81
Q

What is Donath-Landsteiner antibody directed towards?

A

This antibody is directed against the P blood group antigen

82
Q

What is Donath-Landsteiner antibody associated with?

A

associated with tertiary or congenital syphilis and is found after some viral infections

Clinical Hematology (6 decks)

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