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PAS 753 (Molecular Pathophysiology) > hematological disorders > Flashcards

hematological disorders Flashcards

1
Q

what is blood

A

dilute saline with dissolved chemicals and suspended cells

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2
Q

what are four dissolved components of blood

A

nutrients (glucose, vitamins)

Electrolytes (Na, K, Cl, Ca)

Hormones (insulin, T3/T4)

Proteins (albumen, carriers)

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3
Q

T/F drugs are also found dissolved in blood

A

true

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4
Q

differentiate between blood, plasma, and serum

A

blood is all the dissolved and suspended components

plasma is blood with the suspended components removed

serum has all the cell and clotting factors removed

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5
Q

four functional factors of red blood cells

A

Hemoglobin

size, shape

flexibility

longevity

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6
Q

what is the protein structure of hemoglobin

A

a protein tetramer with alpha and beta or alpha and gamma polypeptide chains and one oxygen carrying heme group

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7
Q

what is the structural difference between adult and fetal hemoglobin

A

adult hemoglobin has 2 alpha and 2 beta chains

fetal hemoglobin has 2 alpha and 2 gamma chains

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8
Q

why do RBCs need to flexible?

why is this relevant?

A

RBCs neet to fit through small vessles

as people get older RBCs are less flexible and more likely to clot

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9
Q

what components of blood come from the myeloid line

A

erythrocytes

megakaryocytes (platelets)

granulocytes (N, B, E-phils) and monocytes

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10
Q

what cells come from the lymphoid line

A

NK cells

dendritic cells

T & B lymphoctyes

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11
Q

granular leukocytes

A

basophiles

neutrophils

eosinophils

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12
Q

agranular leukocytes (mononuclear)

A

monocytes

lymphocytes

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13
Q

why do some RBCs appear “speckled”

A

they are reticulocytes that have remains of the endoplasmic reticulum remaining

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14
Q

how long do RBCs last before being broken down

A

100-120 days

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15
Q

what cells break down RBCs

A

reticuloendothelial cells found in the spleen and liver

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16
Q

what is the main byproduct of hemoglobin breakdown

A

bilirubin

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17
Q

bilirubin is an indicator of what

A

liver function

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18
Q

what causes jaundice

A

high levels of bilirubin

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19
Q

T/F a small number of RBCs are lost through the GI, Urine, Skin

A

true

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20
Q

anemia

A

pathological deficiency of oxygen carrying capacity of blood caused by a decrease in the number or function of RBCs

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21
Q

what is a normal blood volume

A

4-8 liters

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22
Q

T/F gamma hemoglobin production is turned off in adults

A

true

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23
Q

white pulp of the spleen

A

lymph tissue

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24
Q

red pulp of the spleen

A

tortuous sinsoids that weed out old RBCs or RBCs with antigens to be broken down

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25
where do RBCs come from does all bone marrow produce RBCs
bone marrow no, mostly in the hips, sternum, long bones
26
erythropoetin
cytokine that stimulates the production of erythrocytes
27
what triggets the production of EPO
decreased oxygen saturation in the blood
28
where is erythropoietin produce
the juxtapoglomerular cells of the kidneys
29
band cells
immature neutrophils that are indicative of rapid production of neutrophils
30
why is neutrophil count important in cancer treatment
chemo decreases bone marrow function
31
Do RBCs have a nucleus? why is that important in transfusion
no because we don;t have to match HLA-1 antigens just blood type
32
what are reticulocytes indictive of
rapid production of RBCs destruction of RBCs
33
recyclining function of RE macrophages
amino acids and iron from hemoglobin is recycled into the bone marrow to make new RBCs
34
CBC
complete blood count (RBCs, WBCs, platelets, hemoglobin and hematocrit)
35
on a CBC the hematocrit is generally how much higher than hemoglobin
3x
36
when you spin out blood what is the "buffy coat"
white blood cells
37
what are the signs of hypervolemia based on the amount of blood lost
10-15% initial signs of vascular instability greater than 30% orthostatic hypertension greater than 40% hypovolemic shock
38
hemoglobin content is dependent on what two factors
production of Hgb - loss of Hgb
39
reduced hgb production is indicative of what?
bone marrow issue
40
increasd hgb loss means
accelerated destruction or loss from vasculature
41
three questions to ask when considering bone marrow deficiency in anemia
enough stem cells enough nutrients to make RBCs (iron, folate) enough stimulation (EPO)
42
three systems that patients will often loss RBCs through (example of disoder)
GYN (heavy period) GI (cancer, ulcer) GU (cancer)
43
four problems that would lead to decreased erythropoesis in the RBCs
nutritional deficiencies loss of stimulation toxicity neoplasm
44
two general nutrition deficiency that cause anemia
anemia of chronic disease starvation
45
three specifict nutrient deficiencies that will cause anemia
folate b12 Iron
46
four dietary sources of iron
meat, seafood, beans, spinach
47
two histological descriptors of iron deficient anemia
microcytic (small) hypochromic (pale colored)
48
T/F pregnancy and lactation can caused anemia through loss of RBCs
false, they cause increased demand
49
four steps to treat iron deficiecy
stop blood loss if there is any improve diet oral iron parenteral iron
50
what is the complication with oral iron supplements
compliance issues due to GI side effects
51
what is the histological sign of B-12 and folate deficency
megaloblastic anemia
52
megaloblastic anemia
Large RBCs, hypersegmented neutrophils caused by impaired DNA synthesis
53
cobalamin
B 12
54
B12 is important to the synthesis of what
nucleic acid synthesis
55
where is B12 found
tightly bound to protein in meat
56
what is needed to extract dietary b12
low gastric pH and intrinsic factor
57
causes of b12 deficiency
dietary (strict vegan) gastric dysfunction GI malabsorption (ileal disease)
58
three ways gastric disfunction can cause b12 deficiency
pernicious anemia gastric atrophy gastric surgery
59
what causes pernicious anemia
auto antibodies against intrinsic factor
60
what will be two signs of pernicious anemia
megaloblastic anemia and progressive neurological deterioration
61
why is it dangerous to give folate to treat pernicious anemia
folate will correct the anemia but not the neurlogical damage
62
treatment for b12 deficiency
large oral doses of B12 intramuscular B12
63
folic acid is an essential cofactor in what
amino acid and DNA synthesis
64
what is the effect of folic acid on fetal development
assist in neural tube development and decreases ther risk of anencephaly or spina bifida
65
causes of folate deficincy
not enough fruits and veggies increased demand (pregnancy, hemolytic anemia) malabsorption of folate
66
folate deficiency treatment
daily folic acid tablets
67
if there is a coexisting B12 deficiency what will folate treatment do
correct anemia but not neurodegeneration
68
how do chronic renal failure cause anemia
loss of EPO resulting in moderate anemia
69
three causes of bone marrow toxicity
pharmacological environmental immunologic
70
three environmental sources of bone marrow toxic substances
solvents pesticides radiation
71
aplastic anemia
an autoimmune disorder that causes the loss of hemopoietic cells
72
what are the three clinical manifestations of aplastic anemia
anemia leukocytopenia (chronic infection) thrombocytopenia (no clotting factors0
73
treatment of aplastic anemia
immunosuppresion bone marrow transplant
74
what is the immunosuppressive treatment of aplastic anemia how often is it successful
antilymphocyte or antithymocyte globulin 50-70%
75
why is a good HLA match needed for bone marrow transplant
a poor match will produce graft versus host disease as the graft produces lymphocytes that react to antigens
76
myeloproliferative disorders
cancers that will displace normal bone marrow
77
types of myeloproliferative disorders
leukemia lymphoma multiple myeloma polycythemia thromocythemia
78
two types of leukemia
myeloid (acute or chronic) lymphoid (acute or chronic)
79
two types of lymphoma
hodgkins non-hodgkins
80
what is the diffence between hodgkins and non-hodgkins
81
polycythemia
overly productive bone marrow that produces too many blood cells
82
risk of thrombocythemia
increased clotting
83
classic GI causes of chronic bleeding
peptic ulcer or cancer
84
classic cause of anemia from gyn
uterine (heavy period, leiomyoma
85
classic urinary causes of anemia
renal cell carcinoma bladder carcinoma
86
causes of hemolysis
defect in RBC size shape or function outside destructrion of normal RBCs
87
hemoglobinopathy two types
change in Hgb structure and function sickle cell anemia thalassemia
88
what causes sickle cell anemia
autosomal recessive mutation of Hgb A
89
what causes sickle cell disease
homozygous vs heterozygous Hgb A mutation
90
T/F heterozygous Hgb is usually asymptomatic
true
91
are the risks associated with sickle cell anemia
chromic hemolytic anemia microinfarction due to clottin
92
what will microinfarctions from sickle cell cause
leg ulcers functional splenomegaly shortened life span
93
thalassemia
genetic defect in globin biosynthesis classified as alpha or beta thalassiema
94
describe the genetic characteristics of thalassemia
autosomal recessive
95
differniate between homozygous and heterozygous thalassemia
homozygotes have signifcant disease (major) heterozygotes are mildly anemia or asymptomatic
96
what are the risks of major thalassemia
severe anemia hepatosplenomegaly growth abnormalities
97
what is the treatment for severe anemia for major thalassemia what is the risk
repeat transfusions iron overload (hemosiderosis)
98
what is the treatment for hepatosplenomegaly caused by thalassemia
eventually splenectomy, which will increase risk of infection
99
why are growth abnormalities present in beta thalassemia
there is an overgrowth of bone marrow that can cause pathologic fractures or "chipmunk facies"
100
two RBC enzyme deficiencies
G6pD PKD
101
T/F G6PD and PKD defiency are both autosomal dominant
false, they are autosomal recessive
102
what is the evolutionary benefit to G6PD deficieny
heterozygotes have increase survival rate with malaria infection
103
G6PD homozygotes are at risk for hemolysis triggered by what three factors
certain diseases (infection, diabetes) certain foods (fava beans) certain drugs (sulfa, aspirin)
104
four other causes of hemolysis that arent sickle cell or
105
important questions to ask in anemai
diet menstruation GI distress medication
106
normochromic, normocytic RBCs with normal reticulocytes, iron but pancytopenia three things that top the DDx
aplastic anemia bone marrow dysfunction drug reaction
107
DDx for megaloblastic anemia low reticulocytes normal iron studies
folate deficiency B12 deficiency metformin GI symptoms
108
DDx for Normal indices (red blood cell size) Reticulocyte count high Iron studies normal Bilirubin level elevated
Hemolysis Inheritied hemoglobin disorderd (thalassemia or sickle cell) Autoimmune disorders (lupus) G6PD Pyruvate kinase deficiency

PAS 753 (Molecular Pathophysiology) (17 decks)

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