Brainscape's Knowledge GenomeTM

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


See full index

T1 - Phase 2 - Systems Modules (I) > Neoplasia/Hematology - Mechanisms of Disease - Nutritional/Hypoproliferative Anemias; Hemolytic Anemias; Transfusion Medicine > Flashcards

Neoplasia/Hematology - Mechanisms of Disease - Nutritional/Hypoproliferative Anemias; Hemolytic Anemias; Transfusion Medicine Flashcards

1
Q

Anemias are typically caused by what three categories of etiology?

A
  1. Blood loss
  2. Underproduction
  3. Destruction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Where is iron absorbed?

Where is folate absorbed?

Where is cobalamin absorbed?

A

Proximal duodenum

proximal jejunum

Terminal ileum

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

What is the normal hemoglobin range for male patients?

A

14 - 16 mg/dL

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

What is the normal hemoglobin range for female patients?

A

12 - 14 mg / dL

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

What is the range of normal hematocrits?

A

36 - 50%

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

What does an MCV measure?

A

The mean corpuscular volume — how large each RBC is

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

What does the MCHC measure?

A

The mean hemoglobin concentration per RBC

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

What does the RDW measure?

A

Cell size uniformity and variability — Is anisocytosis present? Are all the RBCs a similar size?

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

What is the reticulocyte count used to determine?

A

Bone marrow response

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

What is the range of normal MCV values?

A

80 - 100 fL

(normocytic)

(microcytic) < 80 (normocytic) 100 > (macrocytic)

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

Nutritional anemias typically revolve around what main dietary factors?

A

Iron;

B9;

B12

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

A lack of Iron leads to anemia via decreased _______ synthesis.

A lack of B9 leads to anemia via decreased _______ synthesis.

A lack of B12 leads to anemia via decreased _______ synthesis.

A

A lack of Iron leads to anemia via decreased heme synthesis.

A lack of B9 leads to anemia via decreased DNA synthesis.

A lack of B12 leads to anemia via decreased DNA synthesis.

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

Where is iron absorbed into the body?

A

The proximal duodenum

(DMT1 and heme transporters)

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

How much iron is typically lost per day?

A

1 mg

(more during menstruation)

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

Only Fe_?_ is and _______ iron are absorbed.

A

Only Fe2+** (ferrous) and **heme iron are absorbed.

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

Absorbed iron is bound to what in mucosal epithelium?

What transporter allows it to leave the enterocytes to reach the bloodstream?

A

Ferritin;

ferroportin 1

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

What substance is released by the liver in order to block ferroportin 1 (not allowing iron to be absorbed from enterocytes into the bloodstream)?

A

Hepcidin

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

Hepcidin is up-regulated by the liver in response to ______ hepatic iron levels.

A

Hepcidin is up-regulated by the liver in response to increased hepatic iron levels.

(See image — Hepcidin blocks iron uptake from enterocytes to the bloodstream.)

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

Where are the major iron storage sites of the body?

A

The reticuloendothelial system

/

the bone marrow

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

What are the three main serum iron studies?

A
  1. Serum ferritin
  2. ​Serum iron
  3. Total iron-binding capacity (TIBC)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Describe iron deficiency anemia in terms of the following:

RBC count:

H&H:

MCV:

MCHC

RDW:

A

Describe iron deficiency anemia in terms of the following:

RBC count: low

H&H: low

MCV: < 80 fL

MCHC: low

RDW: increased

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

Hypochromic RBCs have a central pallor that is what size of the RBC diameter?

A

> 1/3

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

Are either anisocytosis or poikilocytosis seen in iron deficiency anemia?

A

Both can be seen

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

Describe some of the findings of iron deficiency anemia on peripheral smear.

A

Hypochromic microcytosis;

anisocytosis (increased RDW);

poikilocytosis (elliptocytes, target cells, bizarre forms, etc.)

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

What is the number one cause of anemia within hospitalized patients?

A

Anemia of chronic disease

(anemia of chronic inflammation)

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

What are the typical findings for RBC size in anemia of chronic disease?

A

Micro- or normocytic

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

Anemia of chronic disease is caused by increased levels of IL-___, which leads to increased hepatic _________ synthesis, which leads to decreased iron release from macrophages.

A

Anemia of chronic disease is caused by increased levels of IL-6, which leads to increased hepatic hepcidin synthesis, which leads to decreased iron release from macrophages.

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

Name three acute phase reactants that are upregulated in anemia of chronic disease causing iron sequestration?

A

IL-6;

hepcidin;

ferritin

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

Compare the MCV for the following:

Iron deficiency anemia

Anemia of chronic disease

A

Compare the MCV for the following:

Iron deficiency anemia - decreased

Anemia of chronic disease - decreased or normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Compare the serum iron for the following:

Iron deficiency anemia

Anemia of chronic disease

A

Compare the serum iron for the following:

Iron deficiency anemia — decreased

Anemia of chronic disease — decreased

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

Compare the TIBC for the following:

Iron deficiency anemia

Anemia of chronic disease

A

Compare the TIBC for the following:

Iron deficiency anemia — increased

Anemia of chronic disease — decreased

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

Compare the serum ferritin for the following:

Iron deficiency anemia

Anemia of chronic disease

A

Compare the serum ferritin for the following:

Iron deficiency anemia — decreased

Anemia of chronic disease — increased

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

Compare the marrow stores for the following:

Iron deficiency anemia

Anemia of chronic disease

A

Compare the marrow stores for the following:

Iron deficiency anemia — decreased

Anemia of chronic disease — increased

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

Iron deficiency anemia

MCV: ______

Serum iron: ______

TIBC: ______

Serum ferritin: ______

Marrow stores: ______

A

Iron deficiency anemia

MCV: decreased

Serum iron: decreased

TIBC: increased

Serum ferritin: decreased

Marrow stores: decreased

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

Anemia of chronic disease

MCV: ______

Serum iron: ______

TIBC: ______

Serum ferritin: ______

Marrow stores: ______

A

Anemia of chronic disease

MCV: normal or decreased

Serum iron: decreased

TIBC: decreased

Serum ferritin: increased

Marrow stores: increased

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

The TIBC is basically a measure of what protein?

A

Transferrin

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

What term specifically refers to an anemia characterized by RBCs that are enlarged due to a defect in DNA synthesis?

A

Megaloblastic anemia

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

True/False.

Stress can lead to an elevation in MCV.

A

True.

Due to increased erythropoeisis –> reticulocytosis.

(Reticulocytes are larger than erythrocytes.)

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

What are the two most common categories of megaloblastic anemia?

A

Nutritional (vitamin B9 or B12);

drug-induced

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

Name a few causes of non-megaloblastic macrocytic anemia.

A

Alcoholism;

stress;

liver disease;

hypothyroidism;

myelodysplastic syndromes

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

Name a few medications that can cause a megaloblastic macrocytic anemia.

A

Methotrexate;

hydroxyurea;

zidovudine

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

How do severe cobalamin and/or folate deficiencies appear on peripheral smear?

A

Elevated MCV;

pancytopenia;

low reticulocyte count;

hypersegmented neutrophils;

hyperchromic RBCs

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

Severe cobalamin and/or folate deficiencies result in ______cellular bone marrow.

A

Severe cobalamin and/or folate deficiencies result in hypercellular bone marrow.

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

True/False.

Severe cobalamin and/or folate deficiencies result in hypercellular bone marrow and pancytopenia on peripheral smear.

A

True.

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

Pernicious anemia is characterized by antibodies against what?

A

Intrinsic factor

and/or

parietal cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What term refers to the CNS damage seen in severe B12 deficiencies?

A

Subacute combined degeneration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

B12 deficiencies are sometimes characterized by atrophic _________ and atrophic _________.

A

B12 deficiencies are sometimes characterized by atrophic gastritis and atrophic glossitis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What serum substance is elevated in B12 deficiencies but not B9 deficiencies?

A

Methylmalonic acid

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

In which of the following will folate levels be low?

B12 deficiency

Folate deficiency

B12 and folate deficiency

A

All three!

(B12 is involved in folate synthesis.)

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

Serum ________ levels are increased in both B12 deficiency and folate deficiency.

Serum ________ levels are only increased in B12 deficiency (not folate).

A

Serum homocysteine levels are increased in both B12 deficiency and folate deficiency.

Serum methylmalonic acid levels are only increased in B12 deficiency (not folate).

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

Vitamin B12 is a cofactor for what two enzymes only?

(No others!)

A

Methionine synthase (homocysteine –> methionine)

Methylmalonyl-CoA mutase (MM-CoA to succinyl-CoA)

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

What two cofactors are required for proper methionine synthase activity?

A

B12 and folate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Besides producing methionine from homocysteine, what valuable product necessary to DNA synthesis is produced by the reaction catalyzed by methionine synthase?

A

Tetrahydrofolate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

True/False.

A lack of B12 and/or folate will result in decreased methionine synthase activity, leading to decreased production of tetrahydrofolate, leading to decreased DNA synthesis.

A

True.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Do B12 deficiencies and/or folate deficiencies lead to elevated serum homocysteine?

Do B12 deficiencies and/or folate deficiencies lead to elevated serum methylmalonic acid?

A

Both;

B12 only

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

What is the one major hematologic result of aplastic anemia?

A

Pancytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

____% of cases of aplastic anemia are idiopathic.

A

65% of cases of aplastic anemia are idiopathic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Most (65%) of cases of aplastic anemia are idiopathic.

Name a few drugs associated with this condition.

A

Cloramphenicol;

benzene;

certain chemotherapeutic agents

(leading to a decreased CBC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

What are the two main pathophysiologies seen in aplastic anemia?

A
  1. Autoimmune reaction against bone marrow progenitors
  2. Primary stem cell defect
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Can any infectious agents cause aplastic anemia?

Medical procedures?

Inherited conditions?

A

Some viral infections (e.g. non-A,B,C-type hepatitis);

whole body irradiation;

Fanconi’s anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What is the underlying cause of Fanconi’s anemia?

What is this disorder’s inheritance pattern?

A

Defects in DNA repair;

autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What is the firstline treatment for aplastic anemia in patients under 50?

A

Bone marrow transplant

(immunosuppressives for older individuals)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Name the disease:

a primary bone marrow disorder in which the erythroid progenitors are suppressed.

A

Pure red cell aplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Pure red cell aplasia is associated with what malignancies?

What infectious agent(s)?

Exposure to what drug(s)?

A

Thymomas, LGL leukemias;

parvovirus;

erythropoeitin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

What serum substances are generally elevated in cases of hemolytic anemia?

A

LDH;

bilirubin (indirect and total);

eventually, EPO as well

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What serum substances are generally decreased in cases of hemolytic anemia?

A

Haptoglobin

(binds hemoglobin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What effect does hemolytic anemia generally have on each of the following?

H&H:

Serum total bilirubin:

Serum indirect bilirubin:

A

What effect does hemolytic anemia generally have on each of the following?

H&H: decreased

Serum total bilirubin: increased

Serum indirect bilirubin: increased

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What effect does hemolytic anemia generally have on each of the following?

Serum haptoglobin:

Serum LDH:

Reticulocyte count:

A

What effect does hemolytic anemia generally have on each of the following?

Serum haptoglobin: decreased

Serum LDH: increased

Reticulocyte count: increased

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

The three main ways of classifying a hemolytic anemia:

  1. __________ or __________
  2. Intrinsic or extrinsic
  3. Hereditary or acquired
A

The three main ways of classifying a hemolytic anemia:

  1. Intravascular or extravascular
  2. Intrinsic or extrinsic
  3. Hereditary or acquired
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

The three main ways of classifying a hemolytic anemia:

  1. Intravascular or extravascular
  2. __________ or __________
  3. Hereditary or acquired
A

The three main ways of classifying a hemolytic anemia:

  1. Intravascular or extravascular
  2. Intrinsic or extrinsic
  3. Hereditary or acquired
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

The three main ways of classifying a hemolytic anemia:

  1. Intravascular or extravascular
  2. Intrinsic or extrinsic
  3. __________ or __________
A

The three main ways of classifying a hemolytic anemia:

  1. Intravascular or extravascular
  2. Intrinsic or extrinsic
  3. Hereditary or acquired
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Where does intravascular hemolysis occur?

A

Within circulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Where does extravascular hemolysis occur?

A

The reticuloendothelial system:

Macrophages within the liver, spleen, bone marrow, lymph nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Schistocytes are more closely related to what form of hemolytic anemia?

A

Intravascular hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Microspherocytes are more closely related to what form of hemolytic anemia?

A

Extravascular hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Elevated bilirubin is more closely related to what form of hemolytic anemia?

A

Extravascular hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Elevated free hemoglobin and hemosiderinuria are more closely related to what form of hemolytic anemia?

A

Intravascular hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

True/False.

LDH is typically elevated and haptoglobin is typically decreased in both intravascular and extravascular hemolytic anemias.

A

True.

(Note: haptoglobin may be normal in extravascular hemolytic anemias.)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

All hereditary hemolytic anemias are ___________ (intrinsic/extrinsic).

A

All hereditary hemolytic anemias are intrinsic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

True/False.

All intrinsic hemolytic anemias are hereditary.

A

False.

All hereditary hemolytic anemias are intrinsic to the RBC (but not all intrinsic HAs are inherited).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Name a few examples of hereditary hemolytic disorders.

(Note: all hereditary hemolytic disorders are intrinsic to the RBC, so think about Hgb, membranes, enzymes, etc.)

A

Hgb: Sickle cell, Hgb C, thalassemias

Membranes: Hereditary spherocytosis

Enzymes: G6PD deficiency, PK deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Name a few examples of hereditary hemolytic disorders caused by defects in hemoglobin.

A

Sickle cell, Hgb C, thalassemias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

Name an example hereditary hemolytic disorder caused by membrane defects.

A

Hereditary spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Name a few examples of hereditary hemolytic disorders caused by enzyme defects.

A

G6PD deficiency

Pyruvate kinase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

Is paroxysmal nocturnal hemoglobinuria extrinsic or intrinsic?

Is it hereditary or acquired?

A

Intrinsic,

acquired

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

Name a few general categories of the types of etiologies that might lead to acquired extrinsic hemolyses.

A

Immune;

trauma (e.g. MAHA);

infection (e.g. malaria);

toxins (e.g. lead)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

What does it mean for a hemolytic anemia to be intrinsic?

A

The hemolysis occurs due to a defect in the RBC itself

(e.g. in its hemoglobin, membranes, or enzymes, etc.)

88
Q

What does it mean for a hemolytic anemia to be extrinsic?

A

The hemolysis occurs due to a cause external to the RBCs themselves

(e.g. antibodies, infectious agents, trauma, complement, toxins, etc.)

89
Q

Sickle cell anemia results due to what genetic defect?

A

Glutamate6 change to valine6 in both β-hemoglobin chains

90
Q

True/False.

A single mutation in the gene coding for the sixth glutamic acid in β-hemoglobin is all it takes to result in sickle cell anemia.

A

False.

The disorder is autosomal recessive. A mutation in both genes (SS, homozygous) is necessary.

91
Q

Sickle cell anemia results from a mutation in the __ glutamic acid in β-Hgb to ________.

Hemoglobin C disease results from a mutation in the __ glutamic acid in β-Hgb to ________.

A

6th, valine;

6th, lysine

92
Q

True/False.

Sickle cell vasocclusive crises can affect any organ system.

A

True.

93
Q

What inheritance pattern does sickle cell anemia follow?

A

Autosomal recessive

94
Q

What is the first-line treatment for the disorder shown here?

Via what mechanism does it function?

A

Hydroxyurea;

increasing HgbF concetrations

95
Q

HgbS is most likely to form fibers and sickle RBCs when it is in what state?

What are the implications?

A

The deoxygenated state;

hypoxic conditions (e.g. exercise, high altitudes) can exacerbate the condition

96
Q

Through what mechanism does hydroxyurea improve the signs/symptoms of sickle cell anemia?

A

HgbF levels increase;

HgbF gets incorporated into HgbS chains and halts their continued polymerization

97
Q

Is sickle cell anemia a hemolytic anemia?

A

Yes.

The sickle cells are rigid and inflexible and become jammed and/or lysed in capillary beds

98
Q

Besides hydroxyurea, what other treatments exist for sickle cell anemia in certain situations?

A

Blood transfusions,

prophylactic antibiotics,

pain management,

bone marrow transplants

99
Q

The glutamic acid that is changed to valine in sickle cell anemia is at what amino acid position and in which hemoglobin chain?

The glutamic acid that is changed to lysine in hemoglobin C disease is at what amino acid position and in which hemoglobin chain?

A

6, β-Hgb;

6, β-Hgb

100
Q

What treatments are typically needed for hemoglobin C disease?

A

None;

it is basically a much less severe form of sickle cell anemia

(glutamic acid6 –> lysine6 instead of valine)

101
Q

What form of HbS (Sickle cell hemoglobin) is protective against malaria?

A

The heterozygous form

102
Q

What are the three main types of hemoglobin found in adults?

In what percentages?

A

Adult Hb (HbA) α2β2 >95% of adult Hb

Adult Hb (HbA2) α2δ2 <3% of adult Hb

Fetal Hb (HbF) α2γ2 <3% of adult Hb

103
Q

What are the two forms of adult hemoglobin?

A

HbA α2β2 (>95%)

HbA2 α2δ2

104
Q

True/False.

Individuals with sickle cell anemia are at an increased risk for infection.

A

True.

105
Q

Define thalassemia.

What inheritance pattern do thalassemias follow?

A

A mutation that diminishes or eliminates the production of one of the two chains of hemoglobin;

autosomal recessive

106
Q

What population(s) is most affected by β-thalassemia?

What population(s) is most affected by α-thalassemia?

A

Mediterranean;

Southeast Asian, Middle Eastern, African

107
Q

How are the hemoglobin genes controlled?

On what chromosome are the genes coding for α-globin located?

On what chromosome are the genes coding for β-globin located?

A

by locus control regions;

16;

11

108
Q

In what order are the various β-globin genes organized on chromosome 11?

A

In the order in which they become useful

(starting with the locus control region; epsilon –> gamma –> delta –> beta)

109
Q

True/False.

Thalassemias can be caused by mutations in the coding or regulatory genes for α- or β-globin.

A

True.

110
Q

What is β+-thalassemia?

A

Decreased β-hemoglobin production due to a mutation in the locus control region

111
Q

In what order are the various α-globin genes organized on chromosome 16?

A

In the order in which they become useful

(starting with the locus control region; zeta –> alpha)

112
Q

A newborn presents in the first year of life with severe microcytic hypochromic anemia.

What autosomal recessive disorder might this child have and why are they at an increased risk of heart failure as early as the second or third decade of life?

A

β°-thalassemia major (Cooley’s anemia)

buildup of iron in the heart and other organs

113
Q

A symptomatic patient has both β-hemoglobin genes inactivated. What disorder is this?

An asymptomatic patient has a single β-hemoglobin genes inactivated. What disorder is this?

A symptomatic patient has a single β-hemoglobin genes inactivated. What disorder is this?

A

β°-thalassemia major (Cooley’s anemia);

β-thalassemia minor;

β-thalassemia intermedia

114
Q

What type of α-thalassemia is fatal in-utero and is characterized by virtually no α-globin production?

How many of the 4 α-globin genes (2 alleles each in 2 diploid cells) are deleted in this case?

A

α-thalassemia major (hemoglobin Bart) (hydrops fetalis) (Hgb γ4);

all 4

115
Q

What type of heterozygous, severe α-thalassemia is characterized by only 25% of normal α-globin production?

How many of the 4 α-globin genes (2 alleles each in 2 diploid cells) are deleted in this case?

A

Hemoglobin H disease (Hgb β4 + Hgb γ4)

3

116
Q

Both sickle cell anemia and thalassemias are protective against what?

A

Malaria

117
Q

Is sickle cell anemia best described as a quantitative or qualitative hemoglobinopathy?

A

Qualitative

(defective hemoglobin)

118
Q

Are the thalassemias best described as quantitative or qualitative hemoglobinopathies?

A

Quantiative

(reduced hemoglobin)

119
Q

Name two tests used to screen for sickle cell anemia.

A

Sickle solubility test

Metabisulfite test

120
Q

Name two tests used to confirm sickle cell anemia after screening tests (sickle solubility test, metabisulfite test).

A

Gel electrophoresis (HgbS)

High performance liquid chromatography

121
Q

What is notable about the peripheral smear for a patient with HgbC disease?

A

‘Boxcar’ hemoglobin deposits

122
Q

Describe the general perinatal changes in hemoglobin.

A
123
Q

Thalassemias typically cause ______cytic _____chromic anemias.

A

Thalassemias typically cause microcytic normochromic anemias.

124
Q

What are you likely to see on peripheral smear for thalassemias?

A

Microcytic, normochromic RBCs + target cells;

reticulocytosis

125
Q

What would a CBC likely show for patients with thalassemia?

What RBC count?

MCV?

RDW?

A

Elevated RBC count

decreased MCV

Normal-to-increased RDW

126
Q

What diagnostic test can be used in a patient with suspected hereditary spherocytosis?

A

The osmotic fragility test

(the cells rupture easily in hypotonic solution)

(NOTE: a positive result is not specific to hereditary spherocytosis)

127
Q

What disorder is shown in the attached peripheral smear?

A

Hereditary elliptocytosis

128
Q

G6PD deficiency is associated with __________ anemia due to hemolysis.

A

G6PD deficiency is associated with intermittent anemia due to hemolysis.

129
Q

Pyruvate kinase deficiency is associated with __________ anemia due to hemolysis.

A

Pyruvate kinase deficiency is associated with continuous anemia due to hemolysis.

130
Q

Which enzyme deficiency is associated with hemolytic anemia during times of oxidative stress?

A

G6PD deficiency

131
Q

What is the role of glucose-6-phosphate dehydrogenase in the glycolysis pathway?

A

NONE.

This enzyme is part of the HMP.

132
Q

Why does a G6PD deficiency result in hemolytic anemia?

A

Decreased reduced glutathione and decreased NADPH to buffer against oxidative damage

133
Q

Why does a pyruvate kinase deficiency result in hemolytic anemia?

A

The lack of ATP results in decreased Na/K pump activity (leads to cell dehydration and lysis)

134
Q

What is the inheritance pattern for G6PD deficiency?

A

X-linked recessive

135
Q

Which is typically more severe, G6PD deficiency in Mediterranean populations or African populations?

A

Mediterranean populations

136
Q

G6PD deficiency leads to an increase in oxidized Hgb, leading to formation of what?

A

Heinz bodies

137
Q

Does G6PD deficiency cause intravascular or extravascular hemolysis?

A

Both

138
Q

In patients with G6PD deficiency, the presence of Heinz bodies leads to slower RBC movement through the spleen, leading to what.

A

Bite cells

+

hemolysis

139
Q

What can the following two tests be used to diagnose? (How do they work?)

Ascorbate cyanide

Fluorescent spot

A

G6PD deficiency

Ascorbate cyanide (highly oxidative)

Fluorescent spot (produced NADPH fluoresces)

140
Q

Paroxysmal nocturnal hemoglobinuria:

Mutations in the ______ gene can lead to a decrease in synthesized ______ on RBC surfaces. This leads to decreased inhibition of complement and subsequent RBC hemolysis.

A

Paroxysmal nocturnal hemoglobinuria:

Mutations in the PIG-A gene can lead to a decrease in synthesized PGI on RBC surfaces. This leads to decreased inhibition of complement and subsequent RBC hemolysis.

141
Q

Only _____% of cases of paroxysmal nocturnal hemoglobinuria are paryxosmal and nocturnal.

A

Only 25% of cases of paroxysmal nocturnal hemoglobinuria are paryxosmal and nocturnal.

(Most cases just result in a chronic anemia.)

142
Q

True/False.

Paroxysmal nocturnal hemoglobinuria is best described as a chronic anemia that has associations with leukopenia, thrombocytopenia, thrombosis, and even aplastic anemia, MDS, and AML.

A

True.

143
Q

What is the best test for diagnosing paroxysmal nocturnal hemoglobinuria?

A

Flow cytometry

144
Q

You run flow cytometry on a blood sample from a patient with paroxysmal nocturnal hemoglobinuria. What do you expect to see?

A

A deficiency of CD55 and CD59 on RBCs and neutrophils

145
Q

What two surface markers are missing in paroxysmal nocturnal hemoglobinuria?

A

Decay-accelerating factor (DAF) (CD55);

membrane inhibitor of reactive lysis (MIRL) (CD59)

146
Q

Autoimmune hemolytic anemias can be broken down into what two categories?

A

Warm antibody/agglutinin HA

(or)

cold antibody/agglutinin HA

147
Q

IgG can bind to RBCs at ______ temperatures.

A

IgG can bind to RBCs at warm temperatures.

148
Q

Warm agglutinins (_____ antibodies) typically bind to _____ on RBCs.

A

Warm agglutinins (IgG antibodies) typically bind to Rh factor on RBCs.

149
Q

After warm agglutinins (IgG) bind RBC Rh factor, what happens next to result in hemolytic anemia?

A

Macrophages destroy the RBCs

(via the Fc receptor)

150
Q

What are some examples of diseases associated with autoimmune hemolysis mediated by warm agglutinins (IgG)?

A

Certain infections and malignancies;

collagen vascular disease

151
Q

What are some examples of medications associated with autoimmune hemolysis mediated by warm agglutinins (IgG)?

A

Penicillins, cephalosporins

152
Q

What test can be used to diagnose autoimmune hemolysis mediated by warm agglutinins?

A

Direct Coombs

(add antihuman anti-IgG antibodies to serum)

153
Q

Name some of the characteristic cells you might see on peripheral smear of a person with autoimmune hemolysis mediated by warm agglutinins (IgG).

A

Spherocytes

+

microspherocytes

154
Q

IgM can bind to RBCs at ______ temperatures.

A

IgM can bind to RBCs at cold temperatures.

155
Q

Cold agglutinins (_____ antibodies) typically bind to _____ on RBCs.

A

Cold agglutinins (IgM antibodies) typically bind to I (or i) antigen on RBCs.

156
Q

After cold agglutinins (IgM) bind RBC I antigen, what happens next to result in hemolytic anemia?

A

Phagocytes (in the liver, spleen, and bone marrow) destroy the RBCs via complement-mediated opsonization

157
Q

What are some examples of diseases associated with autoimmune hemolysis mediated by cold agglutinins (IgM)?

A

B cell neoplasms;

certain infectious agents

(e.g. Mycoplasma pneumoniae, EBV, CMV, HIV, etc.)

158
Q

Cold agglutinins are Ig__ that interact with __________ on RBCs.

Warm agglutinins are Ig__ that interact with __________ on RBCs.

A

Cold agglutinins are IgM that interact with I (or i) antigen on RBCs.

Warm agglutinins are IgG that interact with Rh factor on RBCs.

159
Q

Name some of the characteristics you might see on peripheral smear of a person with autoimmune hemolysis mediated by cold agglutinins (IgM).

A

Agglutination

160
Q

What process is described below?

Pathological activation of the clotting cascade –> RBCs lysed as they pass through the fibrin strands

A

Microangiopathic hemolytic anemia

(MAHA)

161
Q

Describe the process of microangiopathic hemolytic anemia:

Pathological activation of the ____________ –> RBCs lysed as they pass through the ___________.

A

Describe the process of microangiopathic hemolytic anemia:

Pathological activation of the clotting cascade –> RBCs lysed as they pass through the fibrin strands.

162
Q

Name a few of the conditions associated with microangiopathic hemolytic anemia (MAHA). Add them to the list below:

________

________

________

pre-eclampsia

prosthetic heart valves

trauma

malignant hypertension

A

TTP

HUS

DIC

pre-eclampsia

prosthetic heart valves

trauma

malignant hypertension

163
Q

Name a few of the conditions associated with microangiopathic hemolytic anemia (MAHA). Add them to the list below:

TTP

HUS

________

________

________

trauma

malignant hypertension

A

TTP

HUS

DIC

pre-eclampsia

prosthetic heart valves

trauma

malignant hypertension

164
Q

Name a few of the conditions associated with microangiopathic hemolytic anemia (MAHA). Add them to the list below:

TTP

HUS

DIC

pre-eclampsia

__________

__________

__________

A

TTP

HUS

DIC

pre-eclampsia

prosthetic heart valves

trauma

malignant hypertension

165
Q

What will be seen on peripheral smear in patients with microangiopathic hemolytic anemia (MAHA) (e.g. due to TTP, DIC, HUS, pre-eclampsia, trauma, etc.)?

A

Schistocytes

166
Q

What is shown in this peripheral smear?

A

Mararial infection

167
Q

Does lead poisoning typically lead to acute or chronic anemia?

A

Chronic

168
Q

Lead poisoning can lead to a deficiency of erythrocyte pyrimidine-specific, 5’-_________.

A

Lead poisoning can lead to a deficiency of erythrocyte pyrimidine-specific, 5’-nucleotidase.

169
Q

What is seen on peripheral smear in a patient with lead poisoning?

A

Basophilic stippling

170
Q

What is the probability that two random individuals are compatible for blood transfusion from one to the other?

A

~65%

171
Q

What are the two most common blood types?

A

O+ (38%)

A+ (34%)

172
Q

What is the universal RBC donor blood type?

A

O-

173
Q

What are the universal plasma donor blood types?

A

AB+

and

AB-

174
Q

Describe what you know about the frequency and features of different blood types.

A
175
Q

What percentage of the world population is Rh+?

A

~85%

176
Q

Are there many RBC antigens important to immunohematology?

A

Yes.

ABO and Rh + ~30 more

177
Q

Pre-Transfusion Testing:

  1. Positive ID on patient and sample
  2. _______________
  3. _______________
A

Pre-Transfusion Testing:

  1. Positive ID on patient and sample
  2. ABO/Rh typing
  3. Check for anti-RBC antibodies
178
Q

What should you order if you don’t suspect a blood transfusion will be necessary for your patient (e.g. you’re taking them into a routine surgery), but you would like the patient’s blood bloodtyped and stored for later cross-matching in it becomes necessary?

A

Order a ‘type and screen’

179
Q

What should you order if you suspect that a blood transfusion will be necessary for your patient, and you would like the following: the patient’s blood to be bloodtyped and tested + compatible units of blood reserved for your patient.

A

Order a ‘type and match’

180
Q

A ‘type and _______’ order is used for patients that may need transfusion, but it is not likely.

A ‘type and _______’ order is used for patients that have a higher likelihood of needing a transfusion.

A

A ‘type and screen’ order is used for patients that may need transfusion, but it is not likely.

A ‘type and match’ order is used for patients that have a higher likelihood of needing a transfusion.

181
Q

How much blood comes in one donor unit?

A

500 mL

182
Q

One unit of donor blood (~500 mL) is equivalent to what percentage of the average patient’s blood volume?

A

12%

183
Q

True/False.

Whole blood is rarely transfused today. Typically component therapy is used so as to only give the patient what they need and also to conserve resources.

A

True.

184
Q

Passing out usually occurs after patients lose about what percentage of their blood volume?

A

~15%

185
Q

What hematocrit does one unit of ADSOL blood have?

A

60%

186
Q

Transfusing one unit of blood into a patient should increase their hemoglobin by ____ mg/dL and their hematocrit by ____%.

A

Transfusing one unit of blood into a patient should increase their hemoglobin by 1** mg/dL and their hematocrit by **3%.

187
Q

On average, adequate oxygen-carrying capacity can be maintained by what minimum hemoglobin concentration?

And hematocrit?

(Of course, age, degree/etiology of anemia, hemodynamic stability, and coexisting cardiovasculopulmonary conditions must be taken into account.)

A

7 g/dL

21%

188
Q

Identify in which of the following scenarios is it appropriate to administer a red cell transfusion:

Volume expansion

Iron replacement

To enhance wound healing

To enhance patient well-being

A

Red cell transfusion is not acceptable in any of these scenarios.

189
Q

True/False.

While platelets have a long shelf-life, RBC units can only be stored for up to 5 days.

A

False.

While RBC units have a long shelf-life, platelets can only be stored for up to 5 days.

190
Q

Why do platelet units for transfusion vary in volume from one to the next?

A

Varying plasma levels

(different volumes collected even though the platelet number remains relatively constant –> the platelets need to ‘off-gas’ H+, and, depending on a number of factors, certain amounts of plasma will be needed to keep the platelets functioning.)

191
Q

Why would you administer a platelet transfusion?

A

To reverse thrombocytopenia and/or platelet dysfunction

–>

to control bleeding

192
Q

True/False.

Platelets should be transfused when any patient’s count has dropped below 20,000/uL.

A

False.

Hospitalized, hemodynamically stable patients can remain clinically stable down to platelet counts as low as 5,000/uL!

(Of course, if not stable and/or if showing oozing/bleeding or other symptoms, then you’ll transfuse.)

193
Q

A leukemia patient with a platelet count of 9,400/uL is presenting with oozing and bleeding. Your first platelet transfusion should be aiming to get their count up to what?

A

50,000/uL

194
Q

1 __________ unit of platelets is derived from many donors and should raise the count by _________/uL.

1 __________ unit of platelets is derived from a single donor and should raise the count by _________/uL.

A

1 random unit of platelets is derived from many donors and should raise the count by 5,000 - 10,000/uL.

1 apheresis unit of platelets is derived from a single donor and should raise the count by 50,000/uL.

195
Q

When should patients with ITP or TTP be administered platelet transfusions?

A

Only if life-threatening bleeding is present

196
Q

What can fresh frozen plasma be used to provide a patient?

A

Clotting factors and inhibitors

(will increase any factor by 3%)

197
Q

Patients with what conditions would be best served by a transfusion of fresh frozen plasma?

A

TTP and HUS

cirrhosis;

also, inherited coagulation factor deficiencies;

reversal of severe warfarin effects

198
Q

True/False.

Patients with less than 75% of normal clotting factors will begin to have bleeding issues.

A

False.

Patients with less than 30% of normal clotting factors will begin to have bleeding issues.

199
Q

Which transfusion unit type is larger, random or apheresis?

A

Apheresis

200
Q

For what general type of condition should red cell units be administered?

A

Severe anemia and/or hemorrhage

201
Q

For what general type of condition should fresh frozen plasma units be administered?

A

Severe clotting factor deficiency and/or severe hemorrhage

202
Q

For what general type of condition should platelet units be administered?

A

Severe thrombocytopenia and/or life-threatening bleeding

203
Q

For what general type of condition should red cell units be administered?

For what general type of condition should fresh frozen plasma units be administered?

For what general type of condition should platelet units be administered?

A

Severe anemia and/or hemorrhage

Severe clotting factor deficiency and/or severe hemorrhage

Severe thrombocytopenia and/or life-threatening bleeding

204
Q

What transfusion unit is mostly used for replacing depleted fibrinogen?

A

Cryoprecipitate

205
Q

What is cryoprecipitate?

A

Plasma proteins that were precipitated out of the plasma at cold temperatures

(e.g. clotting factors, especially fibrinogen)

206
Q

What can be used in the surgical setting for stopping microbleeds in delicate locations where cautery or tying aren’t viable options (e.g. the inner ear, the brain, the eye, etc.)?

A

Fibrin glue

(fibrinogen from cryoprecipitate mixed with bovine thrombin)

207
Q

What clotting factor turns an immature, relatively soft clot into a mature, firm clot?

A

Factor XIII

208
Q

What process allows for separating various blood components from one another (e.g. red cells, plasma, platelets, fibrinogen, immunoglobulins, individual clotting factor types, albumin, granulocytes, Rhogam etc.)?

A

Apheresis

209
Q

What does it mean that transfusion products have undergone leuko-reduction?

A

All cellular immune cells are removed from the products

210
Q

Name some reactions that can occur if WBCs are transfused in blood products.

A

Febrile non-hemolytic transfusion reaction;

transfusion-associated GVHD;

transfusion-related acute lung injury

211
Q

True/False.

Transfusion carries the risk of some level of immunomodulation, such as HLA alloimmunization that can interfere with later transplants.

A

True.

212
Q

How can transfusion-related GVHD be prevented?

A

Irradiation of blood components to be transfused

213
Q

What is the mortality rate of transfusion-associated GVHD (typically only seen in immunocompromised patients)?

A

> 90%

214
Q

FIll in the blanks for patients whose transfusion cellular products must be irradiated:

The _______________

For intra__________ transfusions

For ____________ exchange transfusions

For ____________ disease patients

A

FIll in the blanks for patients whose transfusion cellular products must be irradiated:

The immunocompromised

For intrauterine transfusions

For neonatal exchange transfusions

For Hodgkin’s disease patients

215
Q

Which types of blood transfusion products might need to be irradiated?

A

Only cellular products

(RBCs, platelets, whole blood, etc.)

T1 - Phase 2 - Systems Modules (I) (27 decks)

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