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HEMA LECT1 > Anemia Part 4 > Flashcards

Anemia Part 4 Flashcards

1
Q

WHAT ARE THE FACTORS ASSOCIATED WITH ANEMIA DUE TO IMPAIRED OR DEFECTIVE PRODUCTION

A
  1. Disorders of Iron Metabolism and Heme Synthesis
  2. Bone Marrow Failure
  3. Defects of DNA metabolism
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2
Q

Enumerate the different disorders associated with Iron Metabolism and Heme Synthesis

A

Iron Deficiency Anemia
Sideroblastic Anemia
Anemia of Chronic Disease
Iron overload

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3
Q

Example of condition wherein there is a bone marrow failure

A

Aplastic Anemia

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4
Q

Example of Defects of DNA metabolism

A

Megaloblastic Anemia
Non-megaloblastic anemia

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5
Q

Most common anemia in the US

A

IRON DEFICIENCY ANEMIA

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6
Q

A disorder characterized with insufficient iron for high synthesis

A

IRON DEFICIENCY ANEMIA

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7
Q

A disorder associated with inadequate intake, increases need, impaired absorption or chronic blood loss

A

IRON DEFICIENCY ANEMIA

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8
Q

IRON DEFICIENCY ANEMIA MAY ARISE FROM:

A

Pregnancy
Normal growth
Menstruating women, Blood loss, Intravascular hemolysis
GI disorders (tumors, ulcers, hemorrhoids, aspirin ingestion, alcoholism)
Parasitic infections
March hemoglobinuria (Marching/Runner’s anemia)

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9
Q

Enumerate the different clinical symptoms associated with Iron Deficiency Anemia

A

Fatigue, dizziness, pallor
Pica
Angular cheilosis
Glossitis
Koilonychia

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10
Q

It is an inflammatory condition that causes cracking, crusting, and scaling of the corners of the mouth. Also known as Perleche or angular stomatitis

A

Angular cheilosis

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11
Q

Inflammation of the tongue

A

Glossitis

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12
Q

Koilonychia is also know as __?

A

spoon nails

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13
Q

Also known as spoon nails

A

Koilonychia

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14
Q

Nails with a spoon-shaped dent on them. Usually a sign of iron deficiency

A

Koilonychia

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15
Q

In Iron Deficiency anemia, RBC count, hemoglobin, hematocrit, RBC indices, reticulocyte count are all (increased, decreased)

A

decreased

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16
Q

Iron Deficiency Anemia (LABORATORY FINDINGS)

RDW: ___

A

> 15%

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17
Q

Iron Deficiency Anemia (LABORATORY FINDINGS)

RBC: ____, ________

A

Microcytic, hypochromic

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18
Q

Poikilocytosis present in the laboratory findings of iron deficiency anemia

A

target cells, elliptocytes

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19
Q

chronic bleeding

A

Thrombocytosis

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20
Q

Enumerate the different IRON STUDIES

A

Serum Iron
Total Iron Binding Capacity (TIBC)
Transferrin Saturation (% Saturation)
Serum Ferritin
Free Erythrocyte Protoporphyrin (FEP)

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21
Q

measure of iron bound to transferrin

A

Serum Iron

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22
Q

indirectly measures transferrin concentration by measuring its ability to bind iron

A

Total Iron Binding Capacity (TIBC)

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23
Q

percentage of iron binding sites occupied by iron

A

Transferrin Saturation (% Saturation)

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24
Q

reflection of body’s tissue iron stores

A

Serum Ferritin

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25
Q

measurement of protoporphyrin without iron.

A

Free Erythrocyte Protoporphyrin (FEP)

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26
Q

Measures as zinc protoporphyrin (ZPP) using hematofluorometer’

A

Free Erythrocyte Protoporphyrin (FEP)

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27
Q

Serum Iron is the measure of iron bound to _____

A

transferrin

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28
Q

Free Erythrocyte Protoporphyrin (FEP) is the measurement of _____ without _____

A

protoporphyrin, iron

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29
Q

INADEQUATE INTAKE:

Amount of iron loss per day

A

1mg/day

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30
Q

INADEQUATE INTAKE:

Amount of iron replacement

A

1 mg/day

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31
Q

T/F: Inadequate iron over time will lead to depleted iron stores

A

True

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32
Q

Inadequate iron over time will lead to depleted iron stores and will result to ____

A

Inability to produce Hb

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33
Q

INCREASED NEED happens when iron intake becomes (inadequate, adequate) to meet the needs of expanding erythron

A

inadequate

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34
Q

INCREASED NEED in iron deficiency anemia happens during:

A

Rapid growth (infancy, childhood, adolescence)
Pregnancy

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34
Q

EXAMPLE OF IMPAIRED ABSORPTION IN IRON DEFICIENCY ANEMIA

A

Malabsorption (celiac disease)
Matriptase-2 protein mutation
Decreased stomach acidity
Drugs (stomach acid reducers)

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35
Q

T/F: Excessive loss of Hb in the body will result to RAPID hemorrhage or hemolysis

A

FALSE; slow

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36
Q

Example of Chronic blood loss

A

Heavy menstrual bleeding, fibroid tumors, GI bleeding bec of ulcers and tumors, kidney stones, and PNH

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37
Q

IDA: (choices: Low, High, Normal, Variable)

Serum Iron = ___
Serum Ferritin = ____
TIBC = ___
Transferrin Saturation = ___
FEP/ZPP = ___

A

Low
Low
High
Low
High

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38
Q

Sideroblastic Anemia: (choices: Low, High, Normal, Variable)

Serum Iron = ___
Serum Ferritin = ____
TIBC = ___
Transferrin Saturation = ___
FEP/ZPP = ___

A

High
High
Normal/Low
High
High

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39
Q

Lead Poisoning: (choices: Low, High, Normal, Variable)

Serum Iron = ___
Serum Ferritin = ____
TIBC = ___
Transferrin Saturation = ___
FEP/ZPP = ___

A

Variable
Normal
Normal
Normal
High

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40
Q

ACD/ACI: (choices: Low, High, Normal, Variable)

Serum Iron = ___
Serum Ferritin = ____
TIBC = ___
Transferrin Saturation = ___
FEP/ZPP = ___

A

Low
Normal/High
Low
Low
High

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41
Q

Thalassemia (choices: Low, High, Normal, Variable)

Serum Iron = ___
Serum Ferritin = ____
TIBC = ___
Transferrin Saturation = ___
FEP/ZPP = ___

A

Normal/High
Normal/High
Normal
High/Normal
Normal

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42
Q

What is the new term for ANEMIA OF CHRONIC DISEASE

A

: Anemia of Chronic inflammation

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43
Q

Associated with an increased level of Hepcidin and Inability to use iron

A

ANEMIA OF CHRONIC DISEASE

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44
Q

ANEMIA OF CHRONIC DISEASE is associated with:

A

Infections (HIV, TB)
Inflammation (RA, SLE)
Malignancies (Cancer, Hodgkin Lymphoma)

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45
Q

In ANEMIA OF CHRONIC DISEASE, RBC appears as

A

normocytic, normochromic or slightly microcytic, hypochromic anemia

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46
Q

In ANEMIA OF CHRONIC DISEASE, RBC appears Hemoglobin, serum iron, TIBC are all (increased, decreased)

A

decreased

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47
Q

In ANEMIA OF CHRONIC DISEASE, ESR is (increased, decreased)

A

increased

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48
Q

In ANEMIA OF CHRONIC DISEASE, ferritin _____

A

Normal to increase

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49
Q

Blocks in protoporphyrin pathway

A

SIDEROBLASTIC ANEMIA

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50
Q

Iron overload in bone marrow

A

SIDEROBLASTIC ANEMIA

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51
Q

Example of Hereditary (X-linked, autosomal)

A

Porphyrias

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52
Q

SIDEROBLASTIC ANEMIA:

Acquired:

A

Primary Sideroblastic Anemia (RARS)
Secondary Sideroblastic Anemia

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53
Q

Enumerate the different Secondary Sideroblastic Anemia

A

Lead
Antitubercular drugs
Chloramphenicol
Alcohol
Chemotherapeutic drugs

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54
Q

Erythroblasts with iron deposits surrounding the nucleus

A

Ring/Ringed Sideroblast

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55
Q

Stain used in Ring/Ringed Sideroblast

A

Prussian blue

56
Q

Iron accumulation in mature RBCs

A

Siderocyte/Pappenheimer Body

57
Q

Stain used in Siderocyte/Pappenheimer Body

A

Perl’s Prussian Blue
Wright Stain

58
Q

SIDEROBLASTIC ANEMIA

Appearance of RBC in the peripheral smear

A

Microcytic, Hypochromic

59
Q

Interferes with conversion of aminolevulinic acid (ALA) to porphobilinogen

A

Lead

60
Q

Interferes with incorporation of iron and protoporphyrin

A

Lead

61
Q

Lead inhibis ____

A

pyrimidine 5’-nucleotidase

62
Q

Lead interferes with incorporation of _____ and _____

A

iron; protoporphyrin

63
Q

Other term for lead poisoning

A

Plumbism

64
Q

T/F: Lead poisoning occurs mostly in adults

A

F; children

65
Q

Clinical symptoms of Lead Poisoning (Plumbism)

A

Abdominal pain
Muscle weakness
Gum lead line that forms from blue/black deposit of lead sulfate

66
Q

LEAD:

The conversion of ______ to ______ by ALA dehydratase; the result is the accumulation of _____.

A

aminolevulinic acid (ALA)
Porphobilinogen (PBG)
aminolevulinic acid

67
Q

ALA dehydratase is also called as

A

PBG synthase

68
Q

LEAD

The incorporation of iron into protoporphyrin IX by _____; the result is accumulation of ___ and _____ in the _______.

A

ferrochelatase
iron
protoporphyrin
mitochondria

69
Q

Lab Findings of Lead Poisoning

RBC:
Initially: ______; __________

Chronic exposure: ______; __________

____________

A

normocytic; normochromic

microcytic, hypochromic

Basophilic stippling

70
Q

Urine ALA in lead poisoning is (increased, decreased)

A

increased

71
Q

RBC Protoporphyrin in lead poisoning is (increased, decreased)

A

increased

72
Q

RBC Protoporphyrin is measured using

A

FEP

73
Q

associated color in porphyria

A

purple

74
Q

Group of inherited disorders characterized by impaired protoporphyrin production

A

Porphyria

75
Q

Clinical Symptoms of Porphyria

A

Photosensitivity
Fluorescence in teeth and bone
Psychosis
Wine-red color of urine

76
Q

Enzyme affected in Congenital Erythropoietic Porphyria (CEP) – Gunther’s Disease

A

Uroporphyrinogen III synthase deficiency

77
Q

Enzyme affected in Erythropoietic Protoporphyria (EPP)

A

Ferrochelatase deficiency

78
Q

Enzyme affected in X-linked Erythropoietic Protoporphyria (XLEPP)

A

ALA-synthase (gain-of-function)

79
Q

Inheritance associated with Congenital Erythropoietic Porphyria (CEP) – Gunther’s Disease

A

Autosomal recessive

80
Q

Inheritance associated with Erythropoietic Protoporphyria (EPP)

A

Autosomal recessive

81
Q

Inheritance associated with Erythropoietic Protoporphyria (XLEPP

A

X-linked

82
Q

Clinical Symptoms of Congenital Erythropoietic Porphyria (CEP) – Gunther’s Disease

A

Skin redness
hemolytic anemia
skin photosensitivity
splenomegaly

83
Q

Clinical Symptoms of Erythropoietic Protoporphyria (EPP)

A

Photosensitivity
mild anemia

84
Q

Clinical Symptoms of X-linked Erythropoietic Protoporphyria (XLEPP)

A

Photosensitivity
mild anemia (microcytic, hypochromic)

85
Q

Porphyrins (RBC) in Congenital Erythropoietic Porphyria (CEP) – Gunther’s Disease

A

↑↑↑ Uroporphyrinogen I
↑↑ Coproporphyrinogen I

86
Q

Porphyrins (RBC) in Erythropoietic Protoporphyria (EPP)

A

↑↑↑ Free Protoporphyrin

87
Q

Porphyrins (RBC) in X-linked Erythropoietic Protoporphyria (XLEPP)

A

↑ Free protoporphyrin

88
Q

Most common form of iron overload disease

A

Hereditary Hemochromatosis

89
Q

Hereditary Hemochromatosis is caused by a mutation in ___

A

HFE gene

90
Q

Regulates the amount of iron absorbed from food

A

HFE gene

91
Q

Clinical Symptoms of Hereditary Hemochromatosis

A

Arthritis,
Liver cirrhosis
Congestive heart failure
Impotence
Bronze skin
Diabetes
Thyroid deficiency

92
Q

Treatment for Hereditary Hemochromatosis

A

Phlebotomy

93
Q

Fatal bone marrow failure syndrome

A

APLASTIC ANEMIA

94
Q

APLASTIC ANEMIA has no response in _____

A

erythropoietin

95
Q

Features of APLASTIC ANEMIA

A

Pancytopenia
Reticulocytopenia
Bone Marrow Hypocellularity
Depletion of hematopoietic stem cells

96
Q

Etiology of Aplastic Anemia:

How many % are acquired?

A

80-85%

97
Q

Etiology of Aplastic Anemia:

How many % are inherited?

A

15-20%

98
Q

The Pathophysiology of Bone Marrow Failure includes:

A

Destruction of hematopoietic stem cells as a result of injury by drugs, chemicals, radiation, viruses, or autoimmune mechanisms;

Premature senescence and apoptosis of hematopoietic stem cells as a result of genetic mutations;

Ineffective hematopoiesis caused by stem cell mutations or vitamin B12 or folate deficiency;

Disruption of the bone marrow microenvironment that supports hematopoiesis;

Decreased production of hematopoietic growth factors or related hormones;

Loss of normal hematopoietic tissue as a result of infiltration of the marrow space with abnormal cells.

99
Q

RBC appearance in Aplastic Anemia

A

normocytic, normochromic

100
Q

Hemoglobin, retics, WBC, platelets are all (increased, decreased) in aplastic anemia

A

decreased

101
Q

Hemoglobin level in Aplastic anemia

A

<10 g/dL

102
Q

T/F: Bone Marrow is Severely Hypocellular in aplastic anemia

A

TRUE

103
Q

T/F bone marrow in aplastic anemia is decreased to absent erythroid, granulocytic and megakaryocytic cells

A

TRUE

104
Q

Characterized by aplastic anemia, physical abnormalities and cancer susceptibility

A

Fanconi Anemia

105
Q

Clinical features of Fanconi Anemia

A

Low birth weight
Skin hyperpigmentation (café-au-lait spots)
Short stature

106
Q

Characterized by pancytopenia, mucocutaneous abnormalities, and bone marrow failure

A

Dyskeratosis congenita

107
Q

Characterized by pancreatic insufficiency, cytopenia, skeletal abnormalities, and hematologic malignancies

A

Shwachman-Bodian-Diamond Syndrome

108
Q

Shwachman-Bodian-Diamond Syndrome

Peripheral smear: ________, _______ (%)

A

Neutropenia, pancytopenia (25%)

109
Q

Erythropoietic disorder
May be acquired or inherited

A

PURE RED CELL APLASIA (PRCA)

110
Q

transient erythroblastopenia of childhood
(acquired or inherited)

A

acquired

111
Q

Diamond-blackfan anemia (acquired or inherited)

A

inherited

112
Q

RBC appearance in PRCA

A

Normocytic, normochromic (macrocytic)

113
Q

Reticulocytes in PRCA (increased, decreased)

A

decreased

114
Q

WBC and platelets in PRCA (increased, decreased, normal)

A

normal

115
Q

bone marrow in PRCA

A

Erythroid hypoplasia

116
Q

Associated with Defective DNA synthesis

A

MEGALOBLASTIC ANEMIA

117
Q

MEGALOBLASTIC ANEMIA is Caused by

A

Vitamin B12 (cobalamin) and folate deficiency

118
Q

Peripheral Smear results in MEGALOBLASTIC ANEMIA

A

RBC: macrocytic; normochromic with
Oval macrocytes, teardrops
Howell-Jolly Bodies
nucleated RBC
basophilic stippling
Pappenheimer bodies
Cabot Rings
Hypersegmented neutrophils

119
Q

Hemoglobin, hematocrit, retics count are all (increased, decreased) in MEGALOBLASTIC ANEMIA

A

decreased

120
Q

Lactate dehydrogenase, total and indirect bilirubin, iron are all (increased, decreased) in MEGALOBLASTIC ANEMIA

A

increased

121
Q

Vitamin B12 (Cobalamin) Deficiency is aka

A

Pernicious anemia, Addison’s anemia

122
Q

Causes of Vitamin B12 (Cobalamin) Deficiency

A

Intrinsic factor deficiency
Malabsorption, Diphyllobotrium latum infection, total gastrectomy, total vegetarian diet

123
Q

Example of intrinsic factor deficiency in Vitamin B12 (Cobalamin) Deficiency

A

Autoimmune deficiency
H. pylori infection

124
Q

Clinical findings in Vitamin B12 (Cobalamin) Deficiency

A

Pernicious anemia
Achlorhydria
Weakness and numbness
Sore tongue (glossitis)
GI disorders
CNS problems

125
Q

Hemoglobin, hematocrit, RBC, RBC indices are all (increased, decreased)

A

decreased

126
Q

Peripheral Smear in Vitamin B12 (Cobalamin) Deficiency

A

Macrocytosis, Anisocytosis, Poikilocytosis
Basophilic stipplings
nRBC
Hypersegmented neutrophils
Bone Marrow: dominated with megaloblastic cells

127
Q

Causes of Folic Acid Deficiency

A

Poor dietary intake
Malabsorption syndrome
Pregnancy
Drugs

128
Q

Example of drug that can cause folic acid deficiency

A

Methotrexate

129
Q

Example of drug that can cause folic acid deficiency

A

Methotrexate

130
Q

T/F: The clinical findings of Folic Acid Deficiency
is similar to pernicious anemia (without CNS involvement)

A

True

131
Q

T/F: The clinical findings of Folic Acid Deficiency
is similar to pernicious anemia (without CNS involvement)

A

True

132
Q

What are The Causes of MEGALOBLASTIC

A

Vitamin B12 deficiency
Folic acid deficiency
Pernicious anemia

133
Q

What are The Causes of NON-MEGALOBLASTIC

A

Liver disease
Alcoholism
Bone marrow failure

134
Q

Hypersegmented Neutrophils is (present, absent) in megaloblastic

A

present

135
Q

Hypersegmented Neutrophils is (present, absent) in non-megaloblastic

A

absent

136
Q

Shape of Macrocytes in megaloblastic

A

oval

137
Q

Shape of Macrocytes in non-megaloblastic

A

round

138
Q

MCV in megaloblastic

A

≥120 fL

HEMA LECT1 (3 decks)

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