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Systems Pathology II Final > Red Blood Cell Disorders 1 > Flashcards

Red Blood Cell Disorders 1 Flashcards

1
Q

What three things are affected primarily by hematopoietic and lymphoid disorders?

A 
1 RBCs (erythrocytes)
2 WBCs (leukocytes)
3 hemostasis (thrombocytes)
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2
Q

What is hematopoiesis?

A

Formation and development of blood cells, hematology

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3
Q

What is the location of hematopoiesis in children?

A

Marrow of long bones (tibia and femur)

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4
Q

Where does hematopoiesis occur in adults?

A

Pelvis, cranium, vertebral bodies, sternum, ribs

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5
Q

What is the location of extramedullary hematopoiesis?

A

Spleen, liver, lymph nodes, thymus

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6
Q

What is the clinical term for rupture of erythrocytes?

A

Hemolysis

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7
Q

What is released during hemolysis?

A

Hemoglobin (Hb)

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8
Q

What is the clinical term for erythrocyte production?

A

Erythropoiesis

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9
Q

What is the term for an immature RBC?

A

Reticulocyte

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10
Q

Reticulocytes make up what percentage of overall red blood cells?

A

1-3%

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11
Q

What can you assume with a patient who has reticulocytopenia?

A

Marrow failure (decreased circulating reticulocytes)

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12
Q

When do reticulocytes mature?

A

After 1 day in circulation

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13
Q

What is the clinical term for the condition involved increased RBC count?

A

Polycythemia (rare)

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14
Q

What is the prevalence of anemia in the US?

A

4% of men

8% of women

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15
Q

What is the clinical term for the condition involving decreased RBC mass and decreased O2 capacity?

A

Anemia

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16
Q

What are the three main causes of anemia?

A

1 blood loss (hemorrhage)
2 increased RBC destruction (hemolysis)
3 decreased RBC production (marrow failure)

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17
Q

What effect does anemia have on body tissue?

A

Hypoxia (decreased O2 supply)

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18
Q

During anemia, which organ increases its production of erythrocytes?

A

Kidneys (renal fibroblasts release more erythropoietin)

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19
Q

What are the signs and symptoms of anemia?

A

Pallor, fatigue, weakness (lassitude), decreased growth, osseous abnormalities, cachexia

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20
Q

Why does jaundice appear in severe cases of anemia?

A

Hemolysis leads to release of hemoglobin leading to a buildup of bilirubin from the breakdown of heme causing jaundice/gallstones

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21
Q

What is the appearance of RBCs with hemorrhagic anemia?

A

Normocytic, normochromic

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22
Q

What mineral is needed for hemoglobin synthesis?

A

Iron

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23
Q

What effect does chronic blood loss have on iron stores?

A

Decreases it

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24
Q

What are the effects of hemolytic anemia on the body?

A

1 decreased RBC life span (120 days)
2 increased erythropoiesis, extra medullary hematopoiesis, and reticulocytes (up to 8% of blood)
3 retention of RBC debris (iron)

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25
Q

What are the two categories of the effects of hemolytic anemias?

A

1 intracorpuscular defects

2 extracorpuscular defects

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26
Q

Which type of hemolytic anemia is hereditary? Which is acquired?

A 
Hereditary = intracorpuscular defects
Acquired = extracorpuscular defects
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27
Q

How do intracorpuscular defects associated with hemolytic anemia affect RBCs?

A

Enzyme deficiency and disordered hemoglobin synthesis

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28
Q

What things can cause extracorpuscular defects associated with hemolytic anemia?

A

Antibodies, RBC trauma, infections (like malaria)

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29
Q

What is a corpuscle in vascular terminology?

A

Free floating cell

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30
Q

What condition is most likely present if reticulocytes make up 8% of the overall blood?

A

Hemolytic anemia (due to increased erythropoiesis)

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31
Q

What are the two ways in which hemolysis can occur with hemolytic anemia?

A

Intravascular or extravascular

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32
Q

What is the most common way in which hemolysis can occur with hemolytic anemia?

A

Extravascular hemolysis

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33
Q

How does intravascular hemolysis occur?

A

Trauma to RBC membrane

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34
Q

How does extravascular hemolysis occur?

A

Macrophages attacking spleen/liver leading to RBC damage and antibody opsonization

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35
Q

What is the characteristic sign of intravascular hemolysis?

A

Hemoglobin in urine (hemosiderinuria)

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36
Q

What is hemosiderinuria?

A

Dark urine due to presence of hemoglobin

37
Q

What are the signs of extravascular hemolysis?

A

Splenomegaly and jaundice

38
Q

What is the general term for an abnormally shaped RBC?

A

Poikilocyte

39
Q

What is the term for an irregularly shaped RBC post-hemolysis?

A

Schistocyte

40
Q

What is the term for a teardrop-shaped RBC?

A

Dacrocyte

41
Q

What kind of defect is associated with hereditary spherocytosis?

A

Intracorpuscular

42
Q

What is the specific damage involved to the RBC with hereditary spherocytosis?

A

Abnormal RBC membrane (increased fragility)

43
Q

Why is there a decreased life span associated with the RBCs during hereditary spherocytosis?

A

Removed by spleen

44
Q

What is the treatment for hereditary spherocytosis?

A

Partial splenectomy

45
Q

What type of inheritance pattern is involved with hereditary spherocytosis?

A

Autosomal dominant

46
Q

What is the appearance of the RBCs in hereditary spherocytosis?

A

Spherical (spherocytes) and less elastic in nature

47
Q

What particular infection can cause severe aplastic crisis with hereditary spherocytosis?

A

Parvovirus B19 (Fifth disease)

48
Q

Spherocytes lack what appearance seen in normal RBCs?

A

Central pallor

49
Q

What type of inheritance pattern is involved with sickle cell anemia?**

A

Autosomal recessive: beta-globin**

50
Q

What type of anemia is caused by hereditary spherocytosis, sickle cell disease, thalassemia, glucose-6-phosphate dehydrogenase deficiency, paroxysmal nocturnal hemoglobinuria, immunohemolytic anemias, trauma, and malaria?

A

Hemolytic

51
Q

8% of what race are heterozygous “carriers” for sickle cell disease?

A

African Americans

52
Q

What ratio of African Americans are homozygous and HAVE sickle cell disease?

A

1:600

53
Q

What specifically causes the sickling of the RBCs seen in sickle cell disease?

A

Decreased O2 due to microvascular thrombosis

54
Q

Sickle cell formation is common in what kind of areas of the body?

A

Areas of stasis

55
Q

What are the signs and symptoms of sickle cell diseases?

A

Thrombosis, chronic low-level pain, joint pain, priapism, fever, malaise, splenomegaly/infarction, gallstones, decreased growth and osseous distortion

56
Q

What is the acute chest syndrome seen with sickle cell disease?

A

Lung infections/PE due to pulmonary vascular obstruction from reduced O2 causing thrombosis

57
Q

Why are infection and septicemia common among those with sickle cell disease?

A

Patients are functionally asplenic

58
Q

What are four complications seen with sickle cell anemia?

A

1 acute chest syndrome
2 stroke (cerebrovascular obstruction)
3 infection/septicemia
4 hypoxia-inducted fatty changes (from stress)

59
Q

What are the locations of hypoxia-induced fatty changes seen with sickle cells disease?

A

Heart, kidneys, liver

60
Q

Are heterozygous “carriers” of sickle cell disease symptomatic?

A

Rarely

61
Q

What is the prognosis for those homozygous for sickle cell anemia?

A

50% survive beyond 5th decade

62
Q

What are the two most common causes of death in those with sickle cell disease?

A

Acute chest syndrome or stroke

63
Q

What is the most common location of congestion/infarction in sickle cell patients?

A

Bone marrow (can also occur in spleen, liver, kidneys, retina, brain, lungs, or skin)

64
Q

What sign on X-ray is associated with sickle cell anemia?

A

Lincoln log vertebra (H-shaped vertebral bodies due to central depression and microvascular endplate infarctions)

65
Q

Do all sickle cell anemia patients present with Lincoln log vertebra?

A

No, only 10%

66
Q

What type of inheritance pattern is involved with thalassemia?

A

Autosomal recessive

67
Q

What is the appearance of red blood cells with thalassemia?

A

Microcytic and hypochromic

68
Q

What is thalassemia?

A

Abnormal hemoglobin production

69
Q

In what areas of the world do we see a lot of thalassemia?

A

Areas of endemic malaria like Africa or southeastern Asia

70
Q

What are the two types of thalassemia?

A

Beta (chromosome 11) or alpha (chromosome 16)

71
Q

In beta-thalassemia, which globin genes are mutated, and what is the result?

A

Beta; excess alpha-globin

72
Q

In alpha-thalassemia, which globin genes are mutated, and what is the result?

A

Alpha; excess beta-globin

73
Q

What structures are damaged by thalassemia?

A

RBCs (hemolysis) and erythroblasts

74
Q

What are the differences between beta-thalassemia minor and major?

A 
Minor = involves 1 allele and is very mild
Major = involves 2 alleles and leads to sever hemolysis/anemia
75
Q

What condition is also known as beta-thalassemia trait”?

A

Beta-thalassemia minor

76
Q

What is the appearance of RBCs with beta-thalassemia trait?

A

Microcytic and hypochromic (due to decreased hemoglobin formation)

77
Q

Which form of thalassemia does not affect the lifespan of the RBCs?

A

Beta-thalassemia minor

78
Q

How is beta-thalassemia minor diagnosed?

A

Electrophoresis

79
Q

What is the treatment for beta-thalassemia minor?

A

Monitoring serum ferritin and occasional transfusions with iron chelation (to remove excess)

80
Q

What is the treatment for beta-thalassemia major?

A

Repeated transfusions and iron chelation

81
Q

What benefit does treatment for beta-thalassemia major have?

A

Extends survival into 20s and decreases symptoms and deformities

82
Q

What is the eventual cause of death of beta-thalassemia major?

A

Lethal cardiomyopathy from hemochromatosis (iron overload)

83
Q

What may possibly cure beta-thalassemia major?

A

Bone marrow transplant

84
Q

What unique X-ray signs may be seen in those with beta-thalassemia major?

A

“Hair on end” appearance of the skull, lace-like appearance in bones of the hand

85
Q

Which form of thalassemia is less damaging: beta or alpha-thalassemia?

A

Alpha-thalassemia (excess beta-globin is less worse)

86
Q

Why is alpha-thalassemia highly variable in nature?

A

4 total alpha-globin genes that could be affected

87
Q

What condition results from only 1 alpha-globin gene being affected? 2, 3, or all 4?

A 
1 = silent carrier and asymptomatic
2 = alpha-thalassemia trait and asymptomatic
3 = HbH disease which is severe
4 = hydrous fetalis which is lethal in utero without transfusions
88
Q

Alpha-thalassemia trait is similar to which beta-thalassemia condition?

A

Beta-thalassemia minor

89
Q

HbH disease resembles what beta-thalassemia condition?

A

Beta-thalassemia intermedia

Systems Pathology II Final (8 decks)

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