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PHS 3300 > Blood Disorders pt 1 > Flashcards

Blood Disorders pt 1 Flashcards

1
Q

hemoglobinopathies

A

a group of disorders in which there is abnormal production or structure of the hemoglobin molecule.

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2
Q

how does sickle cell disease occur

A

a Glu is replaced with Val in the beta chain of hemoglobin

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3
Q

what is a megaloblast

A

abnormally large erythroid precursors

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4
Q

what is hemolytic anemia

A

RBCs destroyed faster than they can be made

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5
Q

what is aplastic anemia characterized by (3 things)

A

reduction of hematopoietic tissue

fatty marrow replacement

pancytopenia

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6
Q

what are some clinical manifestations of pernicious anemia

A

megaloblastic madness
paresthesia of the feet and hands - proprioception disturbances

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7
Q

what are the symptoms for moderate to severe anemia

A

hypotension
tachynea/dyspnea
tachycardia, transient murmurs, angina, HF

claudication, night cramps
headaches/lightheaded
tinnitus

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8
Q

what are some common causes of anemia

A

blood loss
impaired RBC production
increased RBC destruction

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9
Q

Describe the RBC indices for thalassemia

A

MCV: low
MCH: low
MCHC: norm/dec
Platelets: norm
RBC: inc
WBC: inc
Hb: low
Hct: low

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10
Q

Standard lab values for RBC count

A

M: 4.2-5.4x10^6/mL
F: 3.6-5.0x10^6/mL

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11
Q

Standard lab values for hemoglobin test

A

M: 14-16.5g/dL
F:12-15g/dL

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12
Q

what are some causes of b12 deficiency (3)

A

damage/atrophy of parietal cells in stomach
resectioning of stomach or SI
chronic malabsorption (chrons, aids)

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13
Q

Standard lab values for MCH

A

27-34 pg/cell

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14
Q

what are globin chains

A

the alpha and beta chain aspect of hemoglobin

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15
Q

Petechia

A

small blood vessels leak under skin (under 4mm)

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16
Q

how can we treat aplastic anemia

A

manage symptoms via transfusion
bone marrow transplant
immunosuppression therapy

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17
Q

what is hereditary spherocytosis

A

a disorder of the RBC membrane

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18
Q

Describe the RBC indices for chronic kidney failure

A

MCV: normal/slight dec
MCH: norm/slight dec
MCHC: normal/slight dec
Platelets: decreases as the kidney failure gets worse
RBC: low
WBC: normal/slight inc
Hb: low
Hct: low

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19
Q

genetic aplastic anemia - causes pancytopenia due to defects in DNA repair

A

Fanconi anemia

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20
Q

what are the symptoms for mild to moderate anemia

A

fatigue
weakness
loss of stamina
tachycardia
exertional dyspnea

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21
Q

what causes occlusions with SCD

A

sickled RBCs are not able to pass through membranes and get blocked up

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22
Q

What are some causes of folate deficiency

A

dietary
alcoholism
cirrhosis
pregnancy
malabsorption

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23
Q

what is the treatment for anemia of chronic kidney failure

A

epo therapy

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24
Q

What does the reticulocyte test tell us

A

Percent of immature RBCs

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25
Q

Describe the RBC indices for Sickle Cell

A

lots of different shapes and sizes
Platelets: norm
WBC: norm

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26
Q

what genes is thalassemia associated with

A

mutant genes that suppress the rate of globin chain synthesis

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27
Q

What does the RBC count tell us

A

number of RBCs per mL of blood

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28
Q

B12 and folate are required for what

A

nuclear maturation and DNA synthesis

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29
Q

how do you diagnose aplastic anemia

A

bone marrow biopsy

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30
Q

what are the 2 compensatory mechanisms for hypoxia from anemia

A

increase BF (HR, CO)
restore tissue oxygenation

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31
Q

what hormone stimulates RBC production in response to low pO2

A

erythropoietin

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32
Q

Describe the RBC indices for aplastic anemia

A

MCV: normal
MCH: norm/slight dec
MCHC: normal
Platelets: low
RBC: low
WBC: low
Hb: low

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33
Q

what are two common cardiovascular complications due ot sickle cell disease

A

chamber enlargement
myocardial infarction

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34
Q

What are the terms for a normal and low MCHC

A

normochromic
hypochromic

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35
Q

Purpura

A

small blood vessels leak under skin (4-10mm)

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36
Q

what are 3 clinical manifestations of folate deficiency

A

demeanor change - depression, irritability, sleep deprivation

memory impairment
perversions of smell and taste

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37
Q

Standard lab values for Reticulocyte test

A

1-1.5% of total RBC

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38
Q

what is aplastic anemia caused by

A

toxic, radiant or immunological injury to bone marrow stem cells

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39
Q

Anemia

A

reduction in the total number of erythrocytes/decrease in quality/quantity of hemoglobin

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40
Q

Relative anemia

A

normal total red cell mass with disturbances in regulation of plasma volume

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41
Q

Absolute anemia

A

actual decrease in numbers of RBCs

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42
Q

what causes RBCs to sickle with SCD

A

low O2 levels

43
Q

what causes extrinsic hemolysis

A

drugs, toxins, antibdies, trauma, mechanical factors

44
Q

What does the Mean corpuscular volume test tell us

A

volume of the average RBC

45
Q

what two things do you use to classify hemolytic anemias

A

whether the hemolysis cause is intrinsic or extrinsic

whether hemolysis is inside or outside the vascular compartment

46
Q

what is intravascular hemolysis caused by

A

complement fixation in transfusion reaction
mechanical injuires
toxic factors

47
Q

describe the lab results for someone with thalassemia

A

low Hb
low MCV

48
Q

what is pancytopenia

A

low RBC, WBC and platelets

49
Q

Standard lab values for MCV

A

85-100 fL/RBC

50
Q

why are those with aplastic anemia susceptible to infections

A

neutropenia

51
Q

why are those with aplastic anemia more susceptible to bleeding

A

low levels of platelets

52
Q

what is fanconi anemia

A

genetic aplastic anemia - causes pancytopenia due to defects in DNA repair

53
Q

what ages are most affected by aplastic anemia

A

young 15-25
or old >60

54
Q

what causes extravascular hemolysis

A

RBCs become deformed and cannot transverse capillaries

abnormal RBCs killed by macrophages in the spleen

55
Q

15% of aplastic anemia cases are due to what

A

exposure of high doses of radiation, chemicals and toxins

56
Q

what are the symptoms for mild anemia

A

typically nothing

57
Q

what are the two morphological classifications of anemia

A

according to the MCV and MCHC

58
Q

what does the mean corpuscular hemoglobin tell us

A

Red cell mass

59
Q

what signs does aplastic anemia show

A

petechia and purpura due to low platelet function

60
Q

aplastic anemia is rare, but is the most common form of what kind of anemia? (MCV and MCHC)

A

normocytic
normochromic

61
Q

what are 4 inherited genetic defects causing anemia

A

abnormalities of hb synthesis

inc RBC hemolysis and destruction

structural abnormalities

inherited enzyme deficiencies

62
Q

what are some symptoms are thalassemia minor

A

mild to moderate anemia

63
Q

what is intravascular hemolysis characterized by (4 things)

A

hemoglobinemia
hemoglobinuria
jaundice
hemosiderinuria

64
Q

what would you find in the bone marrow of vitamin deficiency anemia

A

megaloblastic dysplasia

65
Q

What are the terms for a high, normal and low MCV

A

macrocytic
normocytic
microcytic

66
Q

are intrinsic causes of hemolysis hereditary or aquired

A

hereditary

67
Q

what causes intrinsic hemolysis

A

defects of RBC membranes and hemoglobinopathies

68
Q

RBC indices for hereditary spherocytosis

A

MCV: normal/slight dec
MCH: norm/slight dec
MCHC: increased
RBC: norm/dec
WBC: normal
Hb: low
Hct: low

69
Q

what is erythropoietin

A

hormone that stimulates RBC production in response to low pO2

70
Q

what is the treatment for IDA

A

oral iron therapy
iron sulfate - for about 3-6 months

71
Q

is the reticulocyte count for SCD high or low

A

high (immature RBCs)

72
Q

what are some symptoms of major thalassemia

A

bone deformities
hepato/splenomegaly
cardiac failure
hypogonadism (due to excessive intestinal iron absorption)

73
Q

what is the most common and most severe form of the hemolytic anemias

A

sickle cell disease

74
Q

in IDA, RBCs are ____chromic and ______cytic

A

hypochromic
microcytic

75
Q

Describe the RBC indices for B12 or folate related anemia

A

MCV: inc**
MCH: inc
MCHC: normal/slight dec
Platelets: low
RBC: low
WBC: low

76
Q

what are the two groups of RBC disorders

A

Amenia
Polycythemia

77
Q

Standard lab values for MCHC

A

31-35g/dL

78
Q

What does the hematocrit test tell us

A

percent of total blood volume occupied by RBCs

79
Q

what is the difference in the shape and qualities of a RBC in hereditary spherocytosis and normal RBCs

A

spherocytosis:
spherical
rigid
fragile
smaller

80
Q

what are 4 major symptoms of sickle cell disease

A

severe anemia
vessel occlusion
pulm/cardio complications
splenomegaly, hepatomegaly

81
Q

what kind of anemia is sickle cell disease

A

hemolytic

82
Q

are extrinsic causes of hemolysis hereditary or aquired

A

acquired

83
Q

hemolytic anemais are accompanied by what and why

A

jaundice
due to high levels of bilirubin

84
Q

Koilonychia

A

concave, ridged and brittle nails

85
Q

what kind of anemia is thalassemia

A

hemolytic

86
Q

what does an increase in 2,3-DPG do with regards to anemia

A

allows RBCs to release oxygen easier - allows for better oxygenation without increasing CO

87
Q

how is thalassemia classified

A

by the polypeptide chains with deficient synthessis

88
Q

without b12 and folate, what happens

A

DNA synthesis of blast cells is disrupted leading to megaloblasts

89
Q

what is the most common micronutrient deficiency worldwide

A

iron

90
Q

what are 4 specific Iron deficiency anemia symptoms

A

Pallor
Koilonychia
Glossitis
Pica

91
Q

What does the Hemoglobin test tell us

A

number of grams of Hb per liter of blood

92
Q

glossitis

A

bald, fissured tongue appearance

93
Q

what does it mean for something to be hypersegmented

A

have more than the normal number of lobes

94
Q

75% of aplastic anemia cases are due to what

A

autoimmune disease against hematopoietic stem cells

95
Q

what lab results for MCV, MCH, MCHC would you find for vitamin deficiency related anemia

A

increased MCV, MCH
normal MCHC

96
Q

how does chronic kidney failure lead to anemia

A

it leads to impaired EPO production

97
Q

4 anemias related to decreased RBC production

A

aplastic anemia
anemia of chronic renal failiure
vitamin b12 or folate deficiency
iron deficiency anemia

98
Q

Describe the RBC indices for Iron Deficiency Anemia

A

MCV: low
MCH: low
MCHC: low
Platelets: varies
RBC: norm/slight dec
WBC: norm/slight inc
Hb: low
Hct: low

99
Q

what is neutropenia

A

low levels of neutrophils in blood

100
Q

what are the two kinds of aplastic anemia

A

acquired
Familial

101
Q

how can hemolytic anemias be caused

A

congenitally
acquired (immune response)

102
Q

Standard lab values for hematocrit test

A

M:40-50%
F:37-47%

103
Q

pernicious anemia

A

autoimmune disease - antibodies against parietal cells and IF

104
Q

What does the mean corpuscular hemoglobin concentration test tell us

A

grams of hemoglobin per 10mL of RBCs

PHS 3300 (19 decks)

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