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Patho > Chapter 14: RBCs and ETC > Flashcards

Chapter 14: RBCs and ETC Flashcards

1
Q

What is the average content of hemoglobin per red cell measured in picograms

a. mean cell volume
b. mean cell hemoglobin
c. mean cell hemoglobin concentration
d. red cell distribution width
e. hematocrit

A

B

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2
Q

Which of the following is true about hemolytic anemia

a. degradation of hemoglobin accumulation products created as part of the process of red cell hemolysis
b. elevated erythropoietin levels and a compensatory decrease in erythropoiesis
c. decreased number of erythroid precursors in marrow
d. shortened red cell lifespan below the normal 120days

A

D

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3
Q

Intravascular hemolysis is manifested by the following, except:

a. hemoglobinemia
b. hemoglobinuria
c. hemosidenuria
d. hemosiderosis

A

D

Manifestations: anemia, hemoglobinemia, hemoglobinuria, hemosidenuria, jaundice

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4
Q

What is the average concentration of hemoglobin in a given volume of packed red cell measured in g/dL

a. mean cell volume
b. mean cell hemoglobin
c. mean cell hemoglobin concentration
d. red cell distribution width
e. hematocrit

A

C

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5
Q

What is the reduction of the total circulating red cells below normal limits

a. erythrostasis
b. leukemia
c. anemia
d. spherocytosis

A

C

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6
Q

What is the ratio of packed red cells to total blood volume

a. mean cell volume
b. mean cell hemoglobin
c. mean cell hemoglobin concentration
d. red cell distribution width
e. hematocrit

A

E

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7
Q

It is the loss of intravascular volume which can lead to cardiovascular collapse, shock, death, and triggers increased secretion of erythropoietin from kidney

a. acute blood loss
b. chronic blood loss
c. infection of red cells
d. erythropoietin deficiency

A

A

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8
Q

What is an example of complement fixation as a cause of intravascular hemolysis

a. narrowing of microcirculation by thrombi
b. antibodies bind and red cell antigens
c. clastridial sepsis, which results in the release
d. trauma caused by cardiac valves

A

B

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9
Q

What is the coefficient of variation of red cell volume

a. mean cell volume
b. mean cell hemoglobin
c. mean cell hemoglobin concentration
d. red cell distribution width
e. hematocrit

A

D

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10
Q

The following are examples of the clinical features of extravascular hemolysis, except:

a. anemia
b. splenomegaly
c. hemoglobinemia
d. jaundice

A

C

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11
Q

What is the average volume of red cell

a. mean cell volume
b. mean cell hemoglobin
c. mean cell hemoglobin concentration
d. red cell distribution width
e. hematocrit

A

A

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12
Q

It induces anemia when rate of loss exceeds regenerative capacity and leads to depletion of Iron reserves and iron deficiency anemia appears

a. acute blood loss
b. chronic blood loss
c. infection of red cells
d. erythropoietin deficiency

A

B

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13
Q

Which is not a cause of intracellular hemolysis

a. Mechanical injury
b. Complement fixation
c. Extracellular parasites
d. Exogenous Toxic Factors

A

C

Causes:
Mechanical injury
Complement fixation
Intracellular parasites
Exogenous Toxic Factors or Toxic Injury
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14
Q

Which of the following is a morphologic change in hemolytic anemia

a. hemosiderin degradation
b. prominent reticulocytosis
c. extramedullary erythropoiesis
d. cholesterol gallstones

A

B

Morphology:
increased number of erythroid precursors (normoblasts)
prominent reticulocytosis
hemosiderosis (hemosiderin accumulation)
extramedullary hematopoiesis
pigment gallstones (cholelithiasis)
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15
Q

Which of the following is true about hereditary spherocytosis

a. inherited disorder caused by intrinsic defects of red cell membrane skeleton that render red cells spheroid, less deformable, vulnerable to splenic sequestration and destruction
b. autosomal recessive disorder
c. life span of affected red cell decreased on
average 20 to 30 days from normal 120 days
d. reduced skeleton assembly results from the elevated interactions between the membrane proteins

A

A

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16
Q

What is the chief protein component of red cell skeleton?

a. actin
b. myosin
c. spectrin
d. haptoglobin

A

C

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17
Q

Which of the following does not contribute to the abnormalities of hereditary spherocytosis red cells?

a. prolonged splenic exposure
b. increased red cell pH
c. depletion of red cell glucose
d. diminished red cell pH

A

B

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18
Q

What are the small, dark-staining red cells which lack central zone of pallor and is the most specific morphologic finding of HS?

a. spherocytosis
b. reticulocytosis
c. hemosiderosis
d. cholithiasis

A

A

Cholithiasis - pigment stones (40-50% of affected)

Moderate splenomegaly (500-1000 grams) resulting from congestion of the cords of Billroth and increased numbers of phagocytes

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19
Q

Which is not considered in the diagnosis of hereditary spherocytosis?

a. family history
b. hematologic findings
c. laboratory evidence
d. physical exam

A

D

20
Q

Which is not a characteristic clinical feature of hereditary spherocytosis?

a. anemia
b. dyslipidemia
c. splenomegaly
d. jaundice

A

B

21
Q

What is the common hereditary hemoglobinopathy caused by a point mutation in beta-globin that promotes polymerization of deoxygenated hemoglobin

a. G6PD Deficiency
b. Sickle Cell Disease
c. Hereditary Spherocytosis
d. Thalassemia

A

B

22
Q

What has a protective effect from Plasmodium falciparum malaria?

a. G6PD Deficiency
b. Sickle Cell Disease
c. Hereditary Spherocytosis
d. Thalassemia

A

A/B

23
Q

What are small nuclear remnants in red cells due to asplenia?

a. Cholithiasis
b. Heinz bodies
c. Hemoglobin
d. Howell Jolly bodies

A

D

24
Q

Which is true about Glucose-6-Phosphate Dehydrogenase Deficiency?

a. A hemolytic disease due to red cell enzyme defects
b. Females are at higher risk for symptomatic disease
c. Abnormalities in hexose monophosphate shunt or glutathione metabolism induce the ability of red cells to protect themselves against oxidative injuries leading to hemolysis
d. Oxidants cause intravascular hemolysis in G6PD-deficient individuals

A

A

25
Q

Which is not true about acute intravascular hemolysis?

a. anemia, hemoglobinemia, hemoglobinuria, and dyslipidemia
b. begins 2 to 3 days after exposure to environmental triggers
c. self limited, as hemolysis ceases when only younger G6PD-replete and red cells remain
d. recovery phase is heralded by reticulocytosis

A

A

26
Q

Which is not true about Heinz bodies?

a. dark inclusions within red cells stained with crystal violet
b. can damage the membrane sufficiently to cause extravascular hemolysis
c. can cause a less severe membrane damage resulting in decreased red cell deformability
d. membrane-bound precipitates

A

B

27
Q

Which of the following is not a result of sickle cell disease

a. red cell distortion
b. hemolytic anemia
c. microvascular obstruction
d. ischemic tissue damage
e. none of the above

A

E

28
Q

Which is not a major pathologic manifestation of sickle cell disease?

a. chronic hemolysis
b. macrovascular occlusions
c. microvascular occlusions
d. tissue damage

A

B

29
Q

In sickle cell anemia, the peripheral blood demonstrates the following, except:

a. irreversibly sickled cells
b. reticulocytosis
c. target cells
d. none of the options

A

D

30
Q

What are the episodes of hypoxic injury and infarction that cause severe pain in the affected region?

a. vaso-occlusive crises
b. priapism
c. sequestration crises
d. aplastic crises
e. hyposthenuria

A

A

Priapism: affects 45% of males after puberty, may lead to hypoxic damage and erectile dysfunction

31
Q

What is the massive entrapment of sickled red cells lead to rapid splenic enlargement, hypovolemia, shock, which occurs in children with intact spleens?

a. vaso-occlusive crises
b. priapism
c. sequestration crises
d. aplastic crises
e. hyposthenuria

A

C

Aplastic crises: infection of red cell progenitors by parvovirus B19, causing transient cessation of erythropoiesis

Hyposthenuria: sickling provoked by hypertonicity in renal medulla

32
Q

What is the most common cause of beta+ thalassemia

a. splice mutations
b. promoter region mutations
c. chain terminator mutations

A

A

33
Q

What is the most common cause of beta0-thalassemia?

a. splice mutations
b. promoter region mutations
c. chain terminator mutations

A

C

34
Q

Impaired beta-globin synthesis results in anemia. What produces “underhemoglobinized” hypochromic, microcytic red cells with subnormal oxygen transport capacity?

a. diminished survival of red cells
b. imbalance in alpha and beta globin synthesis
c. deficit in HbA synthesis
d. ineffective erythropoiesis

A

C

35
Q

What causes anemia to manifest 9 moths after birth wherein hemoglobin switches from HbF to HbA?

a. beta-thalassemia major
b. beta-thalassemia minor
c. beta-thalassemia intermedia
d. alpha-thalassemia

A

A

it is the most common in Mediterrenean countries

36
Q

It is usually asymptomatic and the peripheral blood smear typically shows hypochromia, microcytosis, basophilic stippling, and target cells

a. beta-thalassemia major
b. beta-thalassemia minor
c. beta-thalassemia intermedia
d. alpha-thalassemia

A

B

beta-thalassemia intermedia - severe but does not require regular blood transfusions

alpha-thalassemia - caused by inherited deletions that result in reduced or absent synthesis of alpha globin chains

37
Q

What are the excess, unpaired gamma-globin chains which forms gamma4 tetramers, that is present in newborns with alpha-thalassemia?

a. HbH
b. HbF
c. hemoglobin Barts
d. hemoglobin tetramers

A

C

HbH - present in older children and adults as excess beta-globin chains which form beta4 tetramers

38
Q

What is caused by the deletion 2 alpha globin genes; more common in Asians and some regions in Africa

a. silent carrier state
b. alpha-thalassemia trait
c. hemoglobin H (HbH) disease
d. hydrops fetalis

A

B

silent carrier state - deletion of 1, causes barely detectable reduction in alpha-globin chain

Hemoglobin H (HbH) disease: deletion of 3 alpha-globin genes; most common in Asians; moderately severe anemia

Hydrops fetalis: most severe form of alpha-thalassemia; deletion of 4 genes; fetus show severe pallor, generalized edema, massive hepatosplenomegaly

39
Q

What is an that is essential for synthesis of certain membrane-associated complement regulatory proteins

a. phosphatidylinositol glycan complex A (PIGA)
b. phosphatidylinositol glycan complementation group A gene (PIGA)
c. phospholipidinositol glyogen compliment group A (PIGA)

A

B

40
Q

What is the test that detects antibodies and/or complement on red cells?

a. direct Coombs antiglobulin test
b. indirect Coombs antiglobulin test
c. direct Coombs antibody test
d. indirect Coombs antibody test

A

A

41
Q

What test tests the patient’s serum for its ability to agglutinate commercially available red cells bearing particular defined antigens?

a. direct Coombs antiglobulin test
b. indirect Coombs antiglobulin test
c. direct Coombs antibody test
d. indirect Coombs antibody test

A

B

42
Q

What form constitutes approximately 80% of cases of immunohemolytic anemia which is caused by antibodies that bind stably to red cells at 37C?

a. warm antibody type
b. cold agglutin type
c. cold hemolysin type
d. warm antiglobulin type

A

A

43
Q

Which is responsible for an unusual entity known as paroxysmal cold hemoglobinuria?

a. a. warm antibody type
b. cold agglutin type
c. cold hemolysin type
d. warm antiglobulin type

A

C

cold agglutin type - caused by IgM antibodies that bind to red cells avidly at low temperatures

44
Q

It is a highly characteristic and common to all forms of megaloblastic anemia

a. hyperchromic
b. nuclear hypersegmentation
c. anisocytosis
d. macro-ovalocytes

A

D

45
Q

What is the specific form of megaloblastic anemia which is caused by autoimmune gastritis that impairs production of intrinsic factor?

a. Malabsorption states
b. Intrinsic factor deficiency
c. Gastrectomy
d. Pernicious anemia

A

D

46
Q

Which is not a major cause of anemia of folate deficiency?

a. decreased intake
b. increased requirement
c. increased consumption
d. impaired utilization

A

C

Patho (13 decks)

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