hemopoesis pt 2 Flashcards

(111 cards)

1
Q

Hematopoietic System
responsible for?
components?

A

Blood cell forming system

composed of:
Lymph tissue
Bone Marrow
Red bone marrow
Yellow bone marrow

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2
Q

which marrow repsonsible?

hematopoesis

A

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

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3
Q

when does yellow marrow form

A

about 4 yrs old, will replace all reed aside from that in flat bones

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4
Q

Hematopoietic Bone Marrow locations in the Adult

A

Vertebrae
Ribs
Sternum
Ilia

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5
Q

Erythrocytes
 formed via?
 Formation Regulated by?
 __% of RBCs replaced daily
 Life span?

A

 Erythropoiesis
 Regulated by kidneys-Erythropoietin (released with decreased O2)
 1% of RBCs replaced daily
 Life span-120 days (4 months)

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6
Q

Development of Blood Cells from
Hematopoietic Stem Cell to Mature Cells diagram

A
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7
Q

Reticulocytes
 what is in cytoplasm?
 Normal range:
 Indicator of?
 Reticulocytosis?
 Reticulocyte count should be?

A

 Immature red blood cells
 Reticular network of RNA in cytoplasm
 Normal range: 0.5% to 1.5%
 Indicator of bone marrow activity
 Reticulocytosis – elevated number of reticulocytes in blood
 Reticulocyte count should be appropriate to the clinical situation

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8
Q

stages of erythropoesis?

A
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9
Q

normal values of RBC, platlets, WBC

peripheral blood examination

A

done via phlebotomy
 Erythrocytes – 4.0 – 5.5 million / mm3
 Thrombocytes – 150 - 400 thousand / mm3
 Leukocytes – 5 – 10 thousand / mm3

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10
Q

how should normal RBC appear on blood smear

A

biconcave and 7-8 microns

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11
Q

Hemoglobin and Hematocrit Values for males and females

A

Hematocrit - percent
Males 40 - 54%
Females 37 - 47%

Hemoglobin – grams per deciliter (100ml)
Males 14 - 18
Females 12 - 16

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12
Q

what is serum

A

plasma-clotting factors

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13
Q

blood components, %

A
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14
Q

hematocrit

A

measures RBC V as a % of the total V

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15
Q

RBC count

A

total number RBC in whole blood

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16
Q

hemglobin measure

A

measure Hb in blood which reflect RBCs

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17
Q

leukocytes measure

A

combined total of all types of WBC, can be broken down to individual cell types

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18
Q

Adult Hematopoietic Bone Marrow in the Adult can be used to?

A

examine potential dx: MM, leukemia and metastesis

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19
Q

Bone Marrow Aspiration
and Biopsy

A

Posterior superior iliac crest, sample stained and viewed

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20
Q

Hemoglobin strucutre components

A

heme and globin

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21
Q

Heme

A

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

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22
Q

forms?

Globin

A

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

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23
Q

normal adult RBC contain which form Hb mainly

A

HbA

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24
Q

what must Fe charge be in Hb

A

2+

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25
Terminology for Reduction in the Number of Cells Erythrocytes (Leukocytes (Thrombocytes )
Erythrocytes – anemia, erythropenia (Leukocytes – leukopenia) (Thrombocytes - thrombocytopenia)
26
Descriptive Terms for Erythrocytes
cell size and hb content (chromic)
27
# causes? RBC cell size classes
Normocytic Macrocytic – B12, Folate deficiency Microcytic – Iron deficiency
28
Hemoglobin content classes
Normochromic Hypochromic
29
most common anemia seen?
Fe def- microcytic hypochromic
30
Anemia  A reduction in?  oxygen carrying capacity?
 A reduction in the erythron – a reduction in the total red cell mass below normal limits  Reduction in the oxygen carrying capacity of the blood leading to tissue hypoxia
31
anemia diagnosed based on?
 Usually diagnosed based on: Inadequate numbers of erythrocytes (low hematocrit - the ratio of packed red cells to total blood volume) Inadequate level of hemoglobin – the hemoglobin concentration of the blood
32
anemias can be due to a disbalance btwn?
RBC production and destruction
33
3 ways anemias occur
Increased RBC destruction Decreased RBC production Blood loss
34
Clinical Features of Anemia skin energy breathing cardio nails tongue Cognitive  extremities  Headache
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
35
why fatique with anemia?
a person with a low hematocrit cannot carry enough oxygen in the blood to meet energy demands. Weakness, malaise, and easy fatigability.
36
why tachycarida with anemia?
Increased heart rate - compensates for the low oxygen carrying capacity of the blood
37
why SOB with anemia
 Shortness of breath / increased respiratory rate – compensates for the poor delivery of oxygen to the tissues. Dyspnea on mild exertion.
38
why low bp with anemia
 Low blood pressure – a decrease in blood viscosity directly lowers total peripheral resistance to the flow of blood, thus lowering mean arterial blood pressure
39
why pale skin with anemia
- 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
40
why headache with anemia
Central nervous system - hypoxia can cause headache, dimness of vision, and faintness
41
hw can anemia lead to cardiac failure
Cardiac failure can develop and compound the tissue hypoxia caused by the deficiency of O2 in the blood
42
Anemias of Increased Blood Destruction
Sickle cell anemia Thalassemia Erythroblastosis fetalis G6PD deficiency Malaria
43
# dx vs trait? genetics? Sickle Cell Anemia
 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
44
HbS homo vs hetero
 Individuals with sickle cell trait (heterozygous for HbS) have a survival advantage in malaria-endemic areas  Homozygous normal – increased mortality due to malaria  Heterozygous HbS – survival advantage  Homozygous HbS – increased mortality due to sickle cell disease  About 8% of African Americans are heterozygous (sickle cell trait)  1 in 600 African Americans are homozygous (sickle cell disease)
45
# dif in hetero? Behavior of HbS in Hypoxic Conditions
 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 – HbA interferes with HbS polymerization in the heterozygous condition  Red cells do not sickle except under conditions of profound hypoxia
46
# anemia? vascular? common locations? spleen? Clinical Effects of Sickling in Sickle Cell Anemia
 Hemolytic anemia - chronic hemolysis leading to jaundice and phagocytosis in spleen  Microvascular occlusions - sickle cells becone arrested during transit through the microvasculature  Vaso-occlusive crises (pain crises)-episodes of hypoxic injury and infarction that cause severe pain in the affected region: Commonly involved sites: bone, lung, liver, brain, spleen  Autosplenectomy
47
Clinical Consequences of Splenectomy
Increased susceptibility to infection with encapsulated organisms Pneumococcus pneumoniae and Hemophilus influenzae
48
what can this be seen with?
hair on end radiograph, can be seen with sickle cell anemia and beta thalassemia major
49
what radiogrpahic anomolies can be seen with SCA
hair in end and step ladder trabeculae
50
Thalassemia
Group of inherited diseases Quantitative problem - too few globins synthesized, A or B could be affected Underproduction of normal globin proteins due to mutations in regulatory genes
51
# can lead to? tx thalassemia
Regular transfusions – iron overload – organ damage
52
# bones? spleen? growth? tx? consequences of thalassemia
 Bone deformities – expansion of marrow space  Splenomegaly - splenectomy  Impaired growth  Bone marrow transplantation
53
# genetics? forms? beta thalassemia
Two genes involved in making beta chain (one from each parent) Severity depends on number of affected beta chain genes One gene – beta-thalassemia minor - beta-thalassemia trait= Mild disease Two genes – beta-thalassemia major (Cooley’s anemia)= Severe disease
54
# genetics? forms? Alpha Thalassemia
 Four genes involved in making alpha chain (two from each parent)  Severity depends on number of affected alpha chain genes  One gene – asymptomatic carrier  Two genes – alpha-thalassemia minor - alpha-thalassemia trait= Mild disease  Three genes – hemoglobin H disease= Moderate to severe disease  Four genes – alpha-thalassemia major – (lethal)
55
what can happen in marrow of mid face with thalassemia
compensatory hyperplasia
56
what can be seen radiograpically with thalassemia
hair on end like SCA
57
ABO Blood Group Incompatibility leads to?
ABO mismatch leads to intravascular hemolysis Antibody-coated erythrocytes destroyed by both complement-mediated lysis and by phagocytosis in spleen
58
# Ab, Ag blood groups
59
Blood Type Distribution ABO
Type O – 45% Type A – 42% Type B – 10% Type AB – 3%
60
Blood Type Distribution Rh
Positive - 85% Negative - 15%
61
Erythroblastosis Fetalis
Hemolytic Blood Rh-Mediated Hemolytic Disease of the Newborn
62
# if known mom - and baby + what can be done? Erythroblastosis Fetalis: 1st Pregnancy at Delivery
 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 baby not harmed
63
Erythroblastosis Fetalis: 2nd Pregnancy at Delivery
 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
64
prevention of Erythroblastosis Fetalis
Rhogam: Rhesus Immune Globulin - RhIg Immunoglobulin Administered to Rh-negative women after pregnancies in which they carried Rh-positive fetuses Anti-D antibodies
65
# high levels of what are seen in fetus? can deposit where? Erythroblastosis Fetalis effects in fetus
 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
66
# abnromal cell in periphery? increased? brain? dental? Clinical Features of Erythroblastosis Fetalis seen in fetus?
 Erythroblasts in peripheral blood  Hyperbilirubinemia  Kernicterus (bilirubin encephalopathy) if bilirubin reaches a high levels  Developmental dental defects reported- enamel hypo and staining
67
# genetic, demo, symptoms Glucose-6-Phosphate Dehydrogenase Deficiency
 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)
68
G6PD importance
 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), decreased RBC
69
individuals with what are G6PD def. (aside from mutation)
All individuals with Favism (hemolysis due to Broad Beans(fava)) are G6PD deficient
70
G6PD can give a survival advantage where
endemic malaria environments
71
superficial manifestation of G6PD def
juandice
72
Malaria  caused by? vector/resivoir? Reproduction in? causes? type of anemia? morbidity, mortality?
Malaria 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
73
Anemias of Decreased Red Blood Cell production
Iron deficiency anemia -microcytic, hypochromic Pernicious anemia (B12 deficiency) - macrocytic Folic acid deficiency anemia - macrocytic Aplastic anemia
74
fe def anemia appearence on smear
microcytic and hypochromic
75
Iron Deficiency Anemia  Most common?  Lack of Fe most common?  RBC app  Seen most often in? why?  Treated with?  When in males, suspect what?
 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
76
tongue with fe def
atrophic
77
fe de f anemia most common presentation
middle aged female with profound anemia
78
causes of fe def
Dietary lack Impaired absorption Increased requirement Chronic blood loss
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how can infants be fe def
breat feeding
80
how cna children be fe def
poor diet
81
# males and females how can adults be fe def
Males - peptic ulcer disease Females - menorrhagia or pregnancy
82
# developed vs undeveloped elderly fe def causes
Colonic polyps / colon adenocarcinoma in Western world Hookworm infection in developing world
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Laboratory Measurements of Iron Status
Serum ferritin Total iron binding capacity (TIBC) % saturation Serum iron
84
Serum ferritin measures reflect what?
Serum ferritin – reflects iron stores in bone marrow macrophages and liver
85
Total iron binding capacity (TIBC)
measure of transferrin molecules in blood
86
% saturation –
percent of transferrin molecules bound by iron (nl = 33%)
87
Serum iron
Serum iron – measure of iron in the blood
88
# demo? what is def? mucosa? esphogus? risk of/where? Plummer-Vinson Syndrome
Scandinavian, Northern European women Severe Fe-deficiency anemia Mucosal atrophy - atrophic glossitis Esophageal webs - dysphagia Increased risk for squamous cell carcinoma: Esophagus, Oropharynx, Posterior Oral Cavity
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Macrocytic Anemia forms
Pernicious anemia (B12 deficiency) - macrocytic Folic acid deficiency anemia - macrocytic
90
what is def in pernicious anemia
B12
91
# absorption req? what is formed/absorbed where? Vitamin B12 (Cobalamin) Metabolism
Absorption of vitamin B12 requires intrinsic factor, which is secreted by the parietal cells of the stomach Cobalamin – Intrinsic Factor complex absorbed in the ileum
92
# type of dx? B12 req for? Pernicious Anemia
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
93
food sources b12
milk, poultry, eggs, meat, shellfish
94
B12 roles
maintenence CNS and RBC production
95
tongue w pernicious anemia
atrophic and ulcerative
96
lips/ventral tongue with pernicious anemia
red
97
# folate needed for? folic acid def
Megaloblastic anemia Dietary deficiency of folic acid Folate required for DNA synthesis
98
aplastic anemia
 Marrow aplasia secondary to supression of multipotent myeloid stem cells (erythrocyte, leukocyte and thrombocyte series), resulting in pancytopenia
99
what can cause aplastic anemia
 May be caused by known myelotoxic agents (eg. whole body radiation)  Antineoplastic drugs (alkylating agents, antimetabolites)  Benzene  Chloramphenicol
100
aplastic anemia pathogenesis may involve
T cell attack on myeloid stem cells
101
prognosis aplastic anemia
unpredictable
102
tx aplastic anemia
bone marrow transplant and transfusions
103
anemias of blood loss
Gastrointestinal bleeding Hemoptysis (coughing up blood) Epistaxis (nosebleed) Hematuria (blood in urine) Menstrual blood loss
104
# vomiting? stool? GI bleeding indications
Hematemasis (vomiting blood) Melena (black stool) Hematochezia (red blood in feces).
105
Menstrual blood loss forms
Menorrhagia (excessive bleeding) Metrorrhagia (irregular bleeding).
106
Fecal Occult Blood Test – Stool Guaiac
Screening test for occult bleeding in GI tract
107
# forms? Polycythemia (Erythrocytosis)
 An increase in the RBC mass can be relative or absolute
108
 Relative polycythemia
dehydration – decreased plasma volume with normal red cell mass
109
 Absolute polycythemia
a true increase in red cell mass can be primary or secondary
110
 Primary absolute polycythemia (polycythemia vera)
 Erythropoietin-independent  Acquired, clonal stem cell disorder (a chronic myeloproliferative disorder)
111
# EPO? response to? examples?  Secondary polycythemia
 Erythropoietin-dependent  Compensatory response to tissue hypoxia: Chronic lung disease, Cigarette smoking, Residence at high altitude, Paraneoplastic syndromes