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Pathology > Hemo 2 > Flashcards

Hemo 2 Flashcards

1
Q

Hematopoietic System
– cell forming system

A

Blood

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2
Q

Hematopoietic System
Blood cell forming system
(3)

A

Lymph tissue
Bone Marrow
-Red bone marrow
-Yellow bone marrow
Circulating blood

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3
Q

Hematopoiesis
(2)

A

Process in which red and white blood cells are
produced
Red bone marrow

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4
Q

Hematopoietic Bone
Marrow in the Adult
(4)

A

Vertebrae
Ribs
Sternum
Ilia

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5
Q

Erythrocytes
(3)

A

 Erythropoiesis
 Regulated by kidneys- Erythropoietin
 1% of RBCs replaced daily

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6
Q

Erythrocytes
 Life span

A

120 days (4 months)

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7
Q

Reticulocytes
(3)

A

 Immature red blood cells
 Reticular network of RNA in cytoplasm
 Indicator of bone marrow activity

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8
Q

Reticulocytes
 Normal range:

A

0.5% to 1.5%

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9
Q

Reticulocytosis -

A

elevated number of reticulocytes in blood
-reticulocyte count should be appropriate to the clinical situation

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10
Q

Peripheral Blood Examination:
Phlebotomy (Venipuncture)
 Normal Peripheral Blood Values
 Erythrocytes –
 Thrombocytes –
 Leukocytes –

A

4.0 – 5.5 million / mm3
150 - 400 thousand / mm3
5 – 10 thousand / mm3

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11
Q

Peripheral Blood Smear - RBCs
(2)

A

Biconcave disks – central pallor
7-8 microns diameter

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12
Q

Hematocrit - percent
Males
Females

A

40 - 54%
37 - 47%

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13
Q

Hemoglobin – grams per deciliter (100ml)
Males
Females

A

14 - 18
12 - 16

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14
Q

Serum =

A

Plasma – Clotting Factors

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15
Q

Heme –

A

non-protein portion
Iron porphyrin - 4 pyrrole rings + iron

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16
Q

Globin –

A

protein portion
HbA (Adult Hb) – 2 alpha, 2 beta
HbF (Fetal Hb) – 2 alpha, 2 gamma

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17
Q

Normal adult red cells contain mainly —

A

HbA

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18
Q

Erythrocytes –

A

anemia, erythropenia

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19
Q

(Leukocytes –

A

leukopenia)

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20
Q

(Thrombocytes -

A

thrombocytopenia)

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21
Q

Cell size
(3)

A

Normocytic
Macrocytic – B12, Folate deficiency
Microcytic – Iron deficiency

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22
Q

Hemoglobin content
(2)

A

Normochromic
Hypochromic

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23
Q

 A reduction in the erythron –

A

a reduction in the total red cell mass
below normal limits

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24
Q

Anemia

A

 Reduction in the oxygen carrying capacity of the blood leading to
tissue hypoxia

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25
Q

Anemia
 Usually diagnosed based on:
Inadequate numbers of erythrocytes (low hematocrit -
Inadequate level of hemoglobin –

A

the ratio of packed red cells to total blood volume)
the hemoglobin concentration of the blood

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26
Q

AnemiasThe lifespan of a red blood cell is about — days
Each day, must replace

A

120

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27
Q

Anemia
Increased
Decreased
— loss

A

RBC destruction
RBC production
Blood

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28
Q

Clinical Features of Anemia
(9)

A

Pallor – pale skin and mucosa
Lethargy – lack of energy, fatigue, weakness
Dyspnea – labored breathing, SOB
Tachycardia, arrhythmia, chest pain
Koilonychia - spoon-shaped nails
Atrophic glossitis
Cognitive problems, dizziness
Cold extremities
Headache

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29
Q

Clinical Symptoms of Anemia
(7)

A

 Fatigue
 Increased heart rate
 Shortness of breath / increased respiratory rate
 Low blood pressure
 Pale Skin
 Central nervous system
 Cardiac failure can develop and compound the tissue hypoxia caused by the deficiency of O2 in the blood

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30
Q

 Fatigue –

A

a person with a low hematocrit cannot carry enough oxygen in the blood to meet energy demands.
Weakness, malaise, and easy fatigability.

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31
Q

 Increased heart rate -

A

compensates for the low oxygen carrying capacity of the blood

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32
Q

 Shortness of breath / increased respiratory rate –

A

compensates for the poor delivery of oxygen to the tissues.
Dyspnea on mild exertion.

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33
Q

 Low blood pressure –

A

a decrease in blood viscosity directly lowers total peripheral resistance to the flow of
blood, thus lowering mean arterial blood pressure

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34
Q

 Pale Skin -

A

hemoglobin is bright red when oxygenated and less red when deoxygenated. Because the redness
of skin is due to the redness of blood, the skin of an anemic person (who has less oxygen in the blood) will be
less red (paler) than the average person

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35
Q

 Central nervous system -

A

hypoxia can cause headache, dimness of vision, and faintness

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36
Q

Anemias of Increased Blood Destruction
(8)

A

Anemias of Increased Blood Destruction
Sickle cell anemia
Thalassemia
Erythroblastosis fetalis
Hereditary spherocytosis
G6PD deficiency
Paroxysmal nocturnal
hemoglobinuria
Autoimmune hemolytic
anemia
Mechanical trauma to
red cells
Malaria

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37
Q

Sickle Cell Anemia
(6)

A

 A hemoglobinopathy
 Inherited, mis-sense mutation of beta chain
 A single AA substitution of valine for glutamic acid
 Forms a new, abnormal hemoglobin, Hemoglobin S - HbS
 Sickle cell disease – homozygous HbS
 Sickle cell trait - heterozygous, a less serious condition

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38
Q

HbS
(3)

A

 Individuals with sickle cell trait (heterozygous for HbS) have a survival
advantage in malaria-endemic areas
 About 8% of African Americans are heterozygous (sickle cell trait)
 1 in 600 African Americans are homozygous (sickle cell disease)

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39
Q

Homozygous normal -

A

increased mortality due to malaria

40
Q

Heterozygous HbS –

A

survival advantage

41
Q

Homozygous HbS –

A

increased mortality due to sickle cell disease

42
Q

Behavior of HbS in
Hypoxic Conditions
(4)

A

 HbS molecules polymerize when deoxygenated, forming HbS aggregates
 Cytosol changes from a freely-flowing liquid to a viscous gel
 With continued deoxygenation, HbS aggregates form long, needle-like fibers
that distort the red cell shape
 Sickle cell trait

43
Q

 Sickle cell trait –

A

HbA interferes with HbS polymerization in the heterozygous
condition
 Red cells do not sickle except under conditions of profound hypoxia

44
Q

Clinical Effects of Sickling
in Sickle Cell Anemia
(5)

A

 Hemolytic anemia
 Microvascular occlusions
 Vaso-occlusive crises (pain crises)
 Commonly involved sites: bone, lung, liver, brain, spleen
 Autosplenectomy

45
Q

 Hemolytic anemia -

A

chronic hemolysis – and jaundice – phagocytosis in
spleen

46
Q

 Microvascular occlusions -

A

sickle cells becone arrested during transit
through the microvasculature

47
Q

 Vaso-occlusive crises (pain crises) –

A

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

48
Q

Clinical Consequences of
Splenectomy
(3)

A

Increased susceptibility to infection with
encapsulated organisms
Pneumococcus pneumoniae
Hemophilus influenzae

49
Q

Thalassemia
(5)

A

Thalassos = sea
“Mediterranean” anemia
Group of inherited diseases
Quantitative problem - too few globins synthesized
Underproduction of normal globin proteins due to
mutations in regulatory genes

50
Q

Thalassemia
(6)

A

 Ineffective production of
globin chains
-Alpha globin chains (α
Thalassemia)
-Beta globin chains (β
Thalassemia)
 Regular transfusions – iron
overload – organ damage
 Bone deformities – expansion
of marrow space
 Splenomegaly - splenectomy
 Impaired growth
 Bone marrow transplantation

51
Q

Beta Thalassemia
(4)

A

Two genes involved in making beta chain (one from
each parent)
Severity depends on number of affected beta chain
genes
One gene
Two genes

52
Q

Beta Thalassemia
One gene –

A

beta-thalassemia minor - beta-thalassemia
trait
Mild disease

53
Q

Beta Thalassemia
Two genes –

A

beta-thalassemia major (Cooley’s anemia)
Severe disease

54
Q

Alpha Thalassemia
(2)

A

 Four genes involved in making alpha chain (two from each parent)
 Severity depends on number of affected alpha chain genes

55
Q

Alpha Thalassemia
 One gene –
 Two genes –
 Three genes –
 Four genes –

A

asymptomatic carrier
alpha-thalassemia minor - alpha-thalassemia trait
- Mild disease
hemoglobin H disease
-Moderate to severe disease
alpha-thalassemia major – (lethal)

56
Q

Blood Transfusion Reactions: ABO Blood Group Incompatibility
(2)

A

ABO mismatch leads to intravascular hemolysis
Antibody-coated erythrocytes destroyed by
both complement-mediated lysis and by
phagocytosis in spleen

57
Q

ABO blood groups
Type O –
Type A –
Type B –
Type AB –

A

45%
42%
10%
3%

58
Q

Rh antigen
Positive -
Negative -

A

85%
15%

59
Q

Hemolytic Blood Rh-Mediated
Hemolytic Disease of the Newborn
Antigens on surface of red cells
ABO antigens –
Rh antigen (Rhesus factor) –

A

A, B, AB, O
Rh+, Rh-

60
Q

Classification of blood types:

A

A pos, A neg, etc.

61
Q

Erythroblastosis Fetalis:
1st Pregnancy at Delivery
(5)

A

 Rh- mom
 Rh+ fetus
 Fetal RBCs cross the placenta and
enter the maternal circulation
during birth trauma
 Prophylactic anti-Rh (D) immune
globulin (Rhogam) within 72 hours
of delivery
 Rhogam lyses fetal RBCs in the
maternal circulation – effectively
removing any available antigen,
so the mom doesn’t develop anti-
Rh antibodies

62
Q

Erythroblastosis Fetalis:
2nd Pregnancy at Delivery
(5)

A

 Rh- mom, with anti-Rh from prior
pregnancy
 Rh+ fetus
 Anamnestic response rapidly
produces anti-Rh (IgG)
 Anti-Rh IgG crosses placenta
and lyses fetal RBCs
 Rh-mediated hemolytic disease

63
Q

Rhogam:
Rhesus Immune Globulin - RhIg
(3)

A

Immunoglobulin
Administered to Rh-negative women after pregnancies
in which they carried Rh-positive fetuses
Anti-D antibodies

64
Q

Erythroblastosis Fetalis
(7)

A

 Rh incompatibility
 Hemolytic anemia in utero
 Rh-negative mother develops antibodies against Rh-positive erythrocytes of
fetus
 Antibodies cross placenta and hemolyze fetal erythrocytes
 High levels of bilirubin and biliverdin
 Deposition in developing teeth
 Only primary teeth affected

65
Q

Clinical Features:
Erythroblastosis Fetalis
(5)

A

 Anemia caused by immune destruction of erythrocytes
 Erythroblasts in peripheral blood
 Hyperbilirubinemia
 Kernicterus (bilirubin encephalopathy) if bilirubin reaches a high
levels
 Developmental dental defects reported - Atasu M, Genc A, Ercalik S,
Enamel hypoplasia and essential staining of teeth from
erythroblastosis fetalis, J Clin Pediatr Dent., 22(3):249-52, 1998

66
Q

Glucose-6-Phosphate
Dehydrogenase Deficiency
(7)

A

 X-linked disease; most common human enzyme defect (African-American male population)
 Most are asymptomatic; at risk for non-immune hemolytic anemia upon exposure to oxidative stress
 Oxidative stress: infections, drugs (aspirin)
 G6PD / NADPH / Glutathione pathway - maintains supply of reduced glutathione to scavenge free
radicals (anti-oxidant)
 Red cells sustain damage from oxidizing free radicals (phagocytosed in spleen)
 All individuals with Favism (hemolysis due to Broad Beans(fava)) are G6PD deficient
 Survival advantage in Malaria endemic environments

67
Q

Malaria
(5)

A

Protozoal disease – primarily Plasmodium falciparum
Female Anopheles mosquito vector, human reservoir
Reproduction in red cells – showers of organisms
produce shaking, chills and fever
Hemolytic anemia
High morbidity, mortality

68
Q

Anemias of Decreased
Red Blood Cell Destruction
(4)

A

Iron deficiency anemia -
microcytic, hypochromic
Anemia of chronic
disease
Sideroblastic anemia
Pernicious anemia (B12
deficiency) - macrocytic
Folic acid deficiency
anemia - macrocytic
Aplastic anemia
Myelophthisic anemia

69
Q

Iron Deficiency Anemia
(6)

A

 Most common anemia in US
 Lack of Fe most common nutritional
deficiency in the world
 Microcytic, hypochromic
 Seen most often in females – more iron
lost in menses than replaced by nutrition
 Treated with iron supplements
 When in males, suspect internal
bleeding

70
Q

Iron Deficiency Anemia
Middle-aged female with
profound anemia
 Hb =

A

6.2 (nl: 12-16 g/dl)

71
Q

Iron Deficiency
(4)

A

Dietary lack
Impaired absorption
Increased requirement
Chronic blood loss

72
Q

Iron Deficiency is Usually Caused by
Dietary Lack or Blood Loss
 Infants –

A

breast feeding

73
Q

Iron Deficiency is Usually Caused by
Dietary Lack or Blood Loss
Children

A

poor diet

74
Q

Iron Deficiency is Usually Caused by
Dietary Lack or Blood Loss
Adults
M/F

A

Males - peptic ulcer
disease
Females - menorrhagia or
pregnancy

75
Q

Iron Deficiency is Usually Caused by
Dietary Lack or Blood Loss
Elderly
(2)

A

Colonic polyps / colon
adenocarcinoma in
Western world
Hookworm infection in
developing world

76
Q

Iron Deficiency is Usually Caused by
Dietary Lack or Blood Loss
(3)

A

 Malnutrition, malabsorption,
gastrectomy

77
Q

Serum ferritin –

A

reflects iron stores in bone marrow
macrophages and liver

78
Q

Total iron binding capacity (TIBC) –

A

measure of transferrin
molecules in blood

79
Q

% saturation –

A

percent of transferrin molecules bound by
iron (nl = 33%)

80
Q

Serum iron –

A

measure of iron in the blood

81
Q

Plummer-Vinson Syndrome
(5)

A

Scandinavian, Northern European women
Severe Fe-deficiency anemia
Mucosal atrophy - atrophic glossitis
Esophageal webs - dysphagia
Increased risk for squamous cell carcinoma

82
Q

increased risk for squamous cell carcinoma
(3)

A

Esophagus
Oropharynx
Posterior Oral Cavity

83
Q

Macrocytic Anemia
macrocytic
(2)

A

Pernicious anemia (B12 deficiency)
Folic acid deficiency anemia

84
Q

Absorption of vitamin
B12 requires

A

intrinsic
factor, which is
secreted by the
parietal cells of the
stomach

85
Q

Cobalamin –

A

Intrinsic
Factor complex
absorbed in the ileum

86
Q

Vitamin B12 Deficiency Anemia:
Pernicious Anemia
(5)

A

Autoimmune disease
Not due to dietary deficiency of B12
A form of megaloblastic anemia caused by
autoimmune gastritis and failure of intrinsic factor
production leading to vitamin B12 deficiency
Loss of ability to absorb Vitamin B12
Vitamin B12 (cobalamin) required for normal folate
metabolism and DNA synthesis

87
Q

Folic Acid Deficiency
(3)

A

Megaloblastic anemia
Dietary deficiency of folic acid
Folate required for DNA synthesis

88
Q

Aplastic Anemia
(5)

A

 Marrow aplasia secondary to supression of multipotent myeloid
stem cells (erythrocyte, leukocyte and thrombocyte series),
resulting in pancytopenia
 May be caused by known myelotoxic agents (eg. whole body
radiation)
 Pathogenesis may involve T cell attack on myeloid stem cells
 Prognosis unpredictable
 Transfusion, bone marrow transplant

89
Q

May be caused by known myelotoxic agents (eg. whole body
radiation)
(3)

A

 Antineoplastic drugs (alkylating agents, antimetabolites)
 Benzene
 Chloramphenicol

90
Q

Anemias of Blood Loss
(5)

A

Gastrointestinal bleeding
Hemoptysis (coughing up blood)
Epistaxis (nosebleed)
Hematuria (blood in urine)
Menstrual blood loss

91
Q

Gastrointestinal bleeding
(3)

A

Hematemasis (vomiting blood)
Melena (black stool)
Hematochezia (red blood in feces).

92
Q

Menstrual blood loss
(2)

A

Menorrhagia (excessive bleeding)
Metrorrhagia (irregular bleeding).

93
Q

Fecal Occult Blood Test – Stool Guaiac

A

Screening test for occult bleeding in GI tract

94
Q

Polycythemia (Erythrocytosis)
(3)

A

 An increase in the RBC mass
 Relative polycythemia – dehydration – decreased plasma volume with normal red
cell mass
 Absolute polycythemia – a true increase in red cell mass

95
Q

 Absolute polycythemia – a true increase in red cell mass
 Primary polycythemia (polycythemia vera)
(2)

A

 Erythropoietin-independent
 Acquired, clonal stem cell disorder (a chronic myeloproliferative disorder)

96
Q

 Secondary polycythemia
(2)

A

 Erythropoietin-dependent
 Compensatory response to tissue hypoxia

Pathology (23 decks)

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