Brainscape's Knowledge GenomeTM

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


See full index

Pathology I > Diseases of Erythrocytes > Flashcards

Diseases of Erythrocytes Flashcards

1
Q

what is the hematopoietic system

A

-blood cell forming system
- process in which red and white blood cells are produced

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

what makes up the hematopoietic system

A
  • lymphoid tissue
  • bone marrow: red and yellow
  • circulating blood
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

where does hematopoiesis occur

A

red bone marrow

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

what are the most common sites of hematopoietic bone marrow in adults

A
  • vertebrae
  • ribs
  • sternum
  • iliac
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what regulates erythropoiesis

A

erythropoietin in the kidneys

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

what percentage of RBCs are replaced daily

A

1%

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

what is the lifespan of RBCs

A

120 days

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

what are reticulocytes

A

immature red blood cells
- reticular network of RNA in cytoplasm

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

what is the normal range of reticulocytes

A

0.5% - 1.5%

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

what are reticulocytes an indicator of

A

bone marrow activity

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

what is reticulocytosis

A

elevated number of reticulocytes in blood

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

what are the recognizable stages of erythropoiesis

A
  • hemocytoblast
  • proerythroblast
  • early erythroblast
  • late erythroblast
  • normoblast
  • reticulocyte
  • erythrocyte
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what are the normal peripheral values for erythrocytes

A

4.0-5.5 million/mm^3

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

what are the normal peripheral values for thrombocytes

A

150-400 thousand/mm^3

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

what are the normal peripheral values for leukocytes

A

5-10 thousand / mm^3

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

describe the shape of RBCs

A
  • bioconcave disks - central pallor
  • 7-8 microns diameter
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what is hematocrit in males and females

A
  • males: 40-54%
  • females: 37-47%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is hemoglobin levels in males and females

A
  • males: 14-18
  • females: 12-16
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

how is serum calculated

A

plasma - clotting factors

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

where is a bone marrow aspirration and biopsy done

A

posterior superior iliac crest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what is hemoglobin structure

A
  • heme: non protein portion
  • globin: protein portion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

describe what makes up the heme portion

A
  • iron porphyrin- 4 pyrrole rings + iron
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what makes up the globin portion

A
  • HbA (adult Hb) - 2 alpha, 2 beta
  • HbF (fetal Hb) - 2 alpha, 2 gamma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

normal adult red cells contain mainly:

A

HbA

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

terminology for reduction in the number of erythrocytes, leukocytes, thrombocytes

A
  • anemia, erythropenia
  • leukopenia
  • thrombocytopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

what are the ways to describe the cell size of erythrocytes and what does each mean

A
  • normocytic
  • macrocytic: B12, folate deficiency
  • microcytic- iron deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what are the ways to describe the hemoglobin content for erythrocytes

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

what is anemia

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

what is anemia diagnosed based on

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

what are the clinical features of anemia

A
  • pallor: pale skin and mucosa
  • lethargy: lack of energy
  • dyspnea
  • tachycardia
  • koilonychia- spoon shaped nails
  • atrophic glossitis
  • cognitive problems, dizziness
  • cold extremities
  • headache
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

why do anemic patients experience fatigue

A

patient with low hematocrit cannot carry enough oxygen in the blood to meet energy demands, weakness, malaise, and easy fatiguability

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

why do anemic patients experience increased heart rate

A

compensates for the low oxygen carrying capacity of the blood

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

when do anemic patients experience shortness of breath

A

compensates for the poor delivery of oxygen to the tissues

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

why do anemic patients experience low BP

A
  • a decrease in blood viscosity directly lowers total peripheral resistance to the flow of blood, thus lowering MAP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

why do anemic patients experience 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 will be less red

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

why is the CNS affected in anemic patients

A

hypoxia can cause headache, dimness of vision and faintness

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

what are the anemias of increased blood cell destruction

A
  • sickle cell anemia
  • thalassemia
  • erythroblastosis fetalis
  • hereditary spherocytosis
  • G6PD deficiency
  • paroxysmal noctural hemoglobinuria
  • autoimmune hemolytic anemia
  • mechanical trauma to red cells
  • malaria
38
Q

describe sickle cell anemia

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
  • sickle cell disease - homozygous
  • sickle cell trait - heterozygous is a serious condition
39
Q

describe HbS and the types of sickle cell anemia

A
  • individuals with sickle cell trait have a survival advantage in malarai endemic areas
  • homozygous normal: increased mortality due to malaria
  • heterozygous HbS: survival advantage
  • homozygous HbS: increased mortality due to sickle cell disease
40
Q

what percentage of african americans are heterozygous HbS

A

8%

41
Q

how many african americans are homozygous HbS

A

1 in 600

42
Q

describe the behavior of HbS in hypoxic conditions

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
43
Q

describe the sickle cell trait

A

HbA interferes with HbS polymerization in the heterozygous condition

44
Q

red cells do not sickle except under conditions of:

A

profound hypoxia

45
Q

what are the clinical effects of sickling in sickle cell anemia

A
  • hemolytic anemia
  • microvascular occlusions
  • vaso-occlusive crises
  • autosplenectomy
  • commonly involved oragns: bone, lung, liver, brain , spleen
46
Q

what is hemolytic anemia

A
  • chronic hemolysis
    -jaundice
  • phagocytosis in spleen
47
Q

what are microvascular occlusions

A

sickle cells become arrested during transit through the microvasculature

48
Q

what are vaso-occlusive crises

A

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

49
Q

what are the clinical consequences of splenectomy

A
  • increaed susceptibility to infection with encapsulated organisms
  • pneumococcus pneumoniae
  • hemophilus influenzae
50
Q

what is thalassemia

A
  • mediterranean anemia
  • group of inherited disorders
  • quantitative problem - too few globins synthesized
  • underproduction of normal globin proteins due to mutations in regulatory genes
51
Q

what are the two types of thalassemia

A
  • alpha globin chains: alpha thalassemia
  • beta globin chains: beta thalassemia
52
Q

what clinical symptoms are seen in thalassemia

A
  • regular transfusions cause iron overload and organ damage
  • bone deformities - expansion of marrow spaces
  • splenomegaly- splenectomy
  • impaired growth
  • bone marrow transplantation
53
Q

describe beta thalassemia

A
  • two genes involved in making beta chain
  • severity depends on number of affected beta chain
  • one gene - beta- thalassemia minor - beta - thalassemia trait: mild disease
  • two genes- beta thalasseia major ( Cooley’s anemia): severe disease
54
Q

describe alpha thalassemia

A
  • four genes involved in making alpha chains
  • severity depends on number of affected alpha chain genes
  • one gene - asymptomatic carrier
  • two genes- alpha thalassemia minor - mild disease
  • three genes- hemoglobin H disease: moderate to severe disease
  • four genes- alpha thalassemia major- lethal
55
Q

ABO mismatch leads to:

A

intravascular hemolysis

56
Q

what is the mechanism of action in ABO blood group incompatibility

A
  • antibody coated erythrocytes destroyed by both complement mediated lysis and by phagocytosis in the spleen
57
Q

what percentages of people are Type O, A , B and AB

A
  • O: 45%
    A: 42%
    B: 10%
    AB:3%
58
Q

what percentages of people are Rh positive and negative

A

-positive: 85%
- negative: 15%

59
Q

what is hemolytic blood Rh- mediated hemolytic disease of the newborn called

A

erythoblastosis fetalis

60
Q

describe 1st pregnancy of erythroblastosis fetalis

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
61
Q

describe 2nd pregnancy in erythroblastosis fetalis

A
  • Rh- mom with anti- Rh from prior pregnancy
  • Rh+ fetus
  • anamnestic response rapidly produced anti-Rh (IgG)
  • anti Rh IgG crosses placenta and lyses fetal RBCs
  • Rh mediated hemolytic disease
62
Q

describe Rhogam

A
  • Rhesus immune globulin - Rhlg
  • immunoglobulin
  • administered to Rh negative women after pregnancies in which they carried Rh positive fetuses
  • anti- D antibodies
63
Q

describe erythroblastosis fetalis

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
64
Q

what are the clinical features of erythroblastosis fetalis

A
  • anemia caused by immune destruction of erythrocytes
  • erythroblasts in peripheral blood
  • hyperbilirubinemia
  • kernicterus (bilirubin ecephalopathy) if bilirubin reaches a high level
  • development of dental defects
65
Q

describe Glucose-6- phosphate dehydrogenase deficiency

A
  • X- linked disease: most common human enzyme defect
  • most are asymptomatic, at risk for non- immune hemolytic anemia upon exposure to oxidative stress
  • oxidative stress: infections, drugs
  • G6PD/NADPH/ Glutathione pathway
  • red cells sustain damage from oxidizing free radicals
  • all individuals with favism are G6PD deficient
  • survival advantage in malarais endemic environments
66
Q

what does the G6PD / NADPH /Glutathione pathway do

A

maintains supply of reduced glutathione to scavenge free radicals (anti- oxidant)

67
Q

describe malaria

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

what are the anemias of decreased red blood cell destruction

A
  • iron deficiency anemia- microcytic, hypochromic
  • anemia of chronic disease
  • sideroblastic anemia
  • pernicious anemia - macrocytic
  • folic acid deficiency anemia- macrocytic
  • aplastic anemia
  • myelophthisic anemia
69
Q

describe iron deficiency anemia

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

what is the Hb in iron deficiency anemia

A

6.2

71
Q

what can cause iron deficiency

A
  • dietary lack
  • impaired absorption
  • increased requirement
  • chronic blood loss
72
Q

what causes iron deficiency in infants

A

breast feeding

73
Q

what causes iron deficiency in children

A

poor diet

74
Q

what causes iron deficiency in adults

A
  • males: peptic ulcer disease
  • females- menorrhagia or pregnancy
75
Q

what causes iron deficiency in elderyly

A
  • colonic polyps/colon adenocarcinoma in western world
  • hookworm infection in developing world
76
Q

what can also cause iron deficiency

A

malnutrition, malabsorption, gastrtectomy

77
Q

what are the laboratory measurements of iron status and what does each tell us

A
  • serum ferritin- reflects iron stores in bone marrow macrophages and liver
  • total iron binding capacity (TIBC)- measure of transferring molecules in blood
  • % saturation - percent of transferrin molecules bound by iron (normal is 33%)
  • serum iron- measure of iron in blood
78
Q

describe Plummer Vinson sydrome

A
  • 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
79
Q

which types of anemia are macrocytic anemia

A
  • pernicious anemia (vitamin B12) deficiency
  • folic acid deficiency anemia
80
Q

describe vitamin B12 metabolsim

A
  • absorption of vitamin B12 requires intrinsic factor which is secreted by pariteal cells of the stomach
  • cobalamin- intrinsic factor complex absorbed in the ileum
81
Q

describe vitamin B12 deficiency anemia: pernicious anemia

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 required for normal folate metabolism and DNA synthesis
82
Q

describe folic acid deficiency

A
  • megaloblastic anemia
  • dietary deficiency of folic acid
  • folate required for DNA synthesis
83
Q

describe aplastic anemia and tx

A
  • marrow aplasia secondary to supression of multipotent myeloid stem cells resulting in pancytopenia
  • prognosis unpredictable
  • transfusion, bone marrow transplant is tx
84
Q

what may aplastic anemia be caused by

A
  • myelotoxic agents
  • antineoplastic drugs - alkylating agents, antimetabolites
  • benzene
  • chloramphenicol
85
Q

what is the pathogenesis of aplastic anemia

A

involves T cell attack on myeloid stem cells

86
Q

what are the anemias of blood loss

A
  • GI bleeding: hematemesis, melena (black stool) , hematochezia (red blood in feces)
  • hemoptysis
  • epistaxis ( nose bleed)
  • hematuria
  • menstrual blood loss: menorrhagia (excessive bleeding) , metrorrhagia (irregular bleeding)
87
Q

what is the fecal occult blood test

A

screening test for occult bleeding in GI tract

88
Q

what is polycythemia

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
89
Q

what are the two types of absolute polycythemia

A

primary (polycythemia vera0
secondary

90
Q

describe primary polycythemia

A
  • erythropoietin- independent
  • acquired, clonal stem cell disorder - a chronic myeloproliferative disorder
91
Q

describe secondary polycythemia

A
  • erythropoietin- dependent
  • compensatory response to tissue hypoxia
  • chronic lung disease
  • cigarette cmoking
  • residence at high altitude
  • paraneoplastic syndromes
92
Q
A

Pathology I (24 decks)

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