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PHS 3300 > Blood Disorders pt 1 > Flashcards

Blood Disorders pt 1 Flashcards

(104 cards)

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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
Describe the RBC indices for Sickle Cell
lots of different shapes and sizes Platelets: norm WBC: norm
26
what genes is thalassemia associated with
mutant genes that suppress the rate of globin chain synthesis
27
What does the RBC count tell us
number of RBCs per mL of blood
28
B12 and folate are required for what
nuclear maturation and DNA synthesis
29
how do you diagnose aplastic anemia
bone marrow biopsy
30
what are the 2 compensatory mechanisms for hypoxia from anemia
increase BF (HR, CO) restore tissue oxygenation
31
what hormone stimulates RBC production in response to low pO2
erythropoietin
32
Describe the RBC indices for aplastic anemia
MCV: normal MCH: norm/slight dec MCHC: normal Platelets: low RBC: low WBC: low Hb: low
33
what are two common cardiovascular complications due ot sickle cell disease
chamber enlargement myocardial infarction
34
What are the terms for a normal and low MCHC
normochromic hypochromic
35
Purpura
small blood vessels leak under skin (4-10mm)
36
what are 3 clinical manifestations of folate deficiency
demeanor change - depression, irritability, sleep deprivation memory impairment perversions of smell and taste
37
Standard lab values for Reticulocyte test
1-1.5% of total RBC
38
what is aplastic anemia caused by
toxic, radiant or immunological injury to bone marrow stem cells
39
Anemia
reduction in the total number of erythrocytes/decrease in quality/quantity of hemoglobin
40
Relative anemia
normal total red cell mass with disturbances in regulation of plasma volume
41
Absolute anemia
actual decrease in numbers of RBCs
42
what causes RBCs to sickle with SCD
low O2 levels
43
what causes extrinsic hemolysis
drugs, toxins, antibdies, trauma, mechanical factors
44
What does the Mean corpuscular volume test tell us
volume of the average RBC
45
what two things do you use to classify hemolytic anemias
whether the hemolysis cause is intrinsic or extrinsic whether hemolysis is inside or outside the vascular compartment
46
what is intravascular hemolysis caused by
complement fixation in transfusion reaction mechanical injuires toxic factors
47
describe the lab results for someone with thalassemia
low Hb low MCV
48
what is pancytopenia
low RBC, WBC and platelets
49
Standard lab values for MCV
85-100 fL/RBC
50
why are those with aplastic anemia susceptible to infections
neutropenia
51
why are those with aplastic anemia more susceptible to bleeding
low levels of platelets
52
what is fanconi anemia
genetic aplastic anemia - causes pancytopenia due to defects in DNA repair
53
what ages are most affected by aplastic anemia
young 15-25 or old >60
54
what causes extravascular hemolysis
RBCs become deformed and cannot transverse capillaries abnormal RBCs killed by macrophages in the spleen
55
15% of aplastic anemia cases are due to what
exposure of high doses of radiation, chemicals and toxins
56
what are the symptoms for mild anemia
typically nothing
57
what are the two morphological classifications of anemia
according to the MCV and MCHC
58
what does the mean corpuscular hemoglobin tell us
Red cell mass
59
what signs does aplastic anemia show
petechia and purpura due to low platelet function
60
aplastic anemia is rare, but is the most common form of what kind of anemia? (MCV and MCHC)
normocytic normochromic
61
what are 4 inherited genetic defects causing anemia
abnormalities of hb synthesis inc RBC hemolysis and destruction structural abnormalities inherited enzyme deficiencies
62
what are some symptoms are thalassemia minor
mild to moderate anemia
63
what is intravascular hemolysis characterized by (4 things)
hemoglobinemia hemoglobinuria jaundice hemosiderinuria
64
what would you find in the bone marrow of vitamin deficiency anemia
megaloblastic dysplasia
65
What are the terms for a high, normal and low MCV
macrocytic normocytic microcytic
66
are intrinsic causes of hemolysis hereditary or aquired
hereditary
67
what causes intrinsic hemolysis
defects of RBC membranes and hemoglobinopathies
68
RBC indices for hereditary spherocytosis
MCV: normal/slight dec MCH: norm/slight dec MCHC: increased RBC: norm/dec WBC: normal Hb: low Hct: low
69
what is erythropoietin
hormone that stimulates RBC production in response to low pO2
70
what is the treatment for IDA
oral iron therapy iron sulfate - for about 3-6 months
71
is the reticulocyte count for SCD high or low
high (immature RBCs)
72
what are some symptoms of major thalassemia
bone deformities hepato/splenomegaly cardiac failure hypogonadism (due to excessive intestinal iron absorption)
73
what is the most common and most severe form of the hemolytic anemias
sickle cell disease
74
in IDA, RBCs are ____chromic and ______cytic
hypochromic microcytic
75
Describe the RBC indices for B12 or folate related anemia
MCV: inc**** MCH: inc MCHC: normal/slight dec Platelets: low RBC: low WBC: low
76
what are the two groups of RBC disorders
Amenia Polycythemia
77
Standard lab values for MCHC
31-35g/dL
78
What does the hematocrit test tell us
percent of total blood volume occupied by RBCs
79
what is the difference in the shape and qualities of a RBC in hereditary spherocytosis and normal RBCs
spherocytosis: spherical rigid fragile smaller
80
what are 4 major symptoms of sickle cell disease
severe anemia vessel occlusion pulm/cardio complications splenomegaly, hepatomegaly
81
what kind of anemia is sickle cell disease
hemolytic
82
are extrinsic causes of hemolysis hereditary or aquired
acquired
83
hemolytic anemais are accompanied by what and why
jaundice due to high levels of bilirubin
84
Koilonychia
concave, ridged and brittle nails
85
what kind of anemia is thalassemia
hemolytic
86
what does an increase in 2,3-DPG do with regards to anemia
allows RBCs to release oxygen easier - allows for better oxygenation without increasing CO
87
how is thalassemia classified
by the polypeptide chains with deficient synthessis
88
without b12 and folate, what happens
DNA synthesis of blast cells is disrupted leading to megaloblasts
89
what is the most common micronutrient deficiency worldwide
iron
90
what are 4 specific Iron deficiency anemia symptoms
Pallor Koilonychia Glossitis Pica
91
What does the Hemoglobin test tell us
number of grams of Hb per liter of blood
92
glossitis
bald, fissured tongue appearance
93
what does it mean for something to be hypersegmented
have more than the normal number of lobes
94
75% of aplastic anemia cases are due to what
autoimmune disease against hematopoietic stem cells
95
what lab results for MCV, MCH, MCHC would you find for vitamin deficiency related anemia
increased MCV, MCH normal MCHC
96
how does chronic kidney failure lead to anemia
it leads to impaired EPO production
97
4 anemias related to decreased RBC production
aplastic anemia anemia of chronic renal failiure vitamin b12 or folate deficiency iron deficiency anemia
98
Describe the RBC indices for Iron Deficiency Anemia
MCV: low MCH: low MCHC: low Platelets: varies RBC: norm/slight dec WBC: norm/slight inc Hb: low Hct: low
99
what is neutropenia
low levels of neutrophils in blood
100
what are the two kinds of aplastic anemia
acquired Familial
101
how can hemolytic anemias be caused
congenitally acquired (immune response)
102
Standard lab values for hematocrit test
M:40-50% F:37-47%
103
pernicious anemia
autoimmune disease - antibodies against parietal cells and IF
104
What does the mean corpuscular hemoglobin concentration test tell us
grams of hemoglobin per 10mL of RBCs

PHS 3300 (19 decks)

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