llefebvre01-
what is anemia?
low RBCs
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what are 4 common causes of anemia?
impaired erythrocyte production
blood loss (acute or chronic)
increase erythrocyte destruction
combination of these 3
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what are Sx of anemia?
paler of skin, hyper dynamic state, fatigue
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what are characteristics of B12 deficiency anemia (mechanism, RBC characteristics + primary cause)?
abnormal DNA/RNA synthesis
macrocytic + normochromic
1- congenital
2- intrinsic factor deficiency
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what are characteristics of folate (B9) deficiency anemia (mechanism, RBC characteristics + primary cause)?
abnormal DNA/RNA synthesis
macrocytic + normochromic
1- dietary folate deficiency
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what are characteristics of iron deficiency anemia (mechanism, RBC characteristics + primary cause)?
insufficient Hb synthesis
microcytic + hypochromic
1- chronic blood loss
2- dietary iron deficiency
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what are characteristics of aplastic anemia (mechanism, RBC characteristics + primary cause)?
insufficient erythropoiesis
normocytic + normochromic
1- bone marrow suppression
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what are characteristics of posthemmorhagic anemia (mechanism, RBC characteristics + primary cause)?
significant blood loss
normocytic + normochromic
1- bleeding
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what are characteristics of hemolytic anemia (mechanism, RBC characteristics + primary cause)?
premature destruction of RBC
normocytic + normochromic
1- increased fragility of RBCs
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what are characteristics of sickle cell anemia (mechanism, RBC characteristics + primary cause)?
abnormal hemoglobin synthesis
normocytic + normochromic
1- congenital
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what is the pathophysiology of B12-deficiency anemia?
pernicious = highly injurious/destructive
absence of intrinsic factor
- parietal cell destructions (congenital; autoimmune)
- stomach or distal ileum surgical removal
- malnutrition
b12 binds to intrinsic factor
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what are the manifestations of B12-deficiency anemia?
common anemia Sx (slow onset of 20-30 years)
specific Sx: demyelination -> neuronal death (not reversible)
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what is the pathophysiology of folate deficiency anemia?
more common than B12
folate = essential for DNA/RNA synthesis
most common causes:
1- malnutrition -> often related to alcholism
2- folate malabsorption -> ex: IBS
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what are the manifestations of folate-deficiency anemia?
ulcerations + inflammation of bucco-oral area
no neurological manifestations
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what is the pathophysiology of iron deficiency anemia?
most common 20% of all cases -> females higher risk cuz of pregnancy + menses
common cause in developed countries -> pregnancy + chronic blood loss
2 main ethologies:
1- inadequate dietary intake
2- excessive blood loss
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what are the causes + manifestations of aplastic anemia?
bone marrow suppression
stem cell environment issues
classic anemia Sx
thrombocytopenia
leukopenia
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what are the causes + manifestations of posthemorrhagic anemia?
any significant bleeding
initial Sx masked by cardiovascular adaptations to hemorrhage
severe shock if blood loss > 2000 mL
fatal lactic acidosis
can lead to IDA
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what are the causes + manifestations of hemolytic anemia?
autoimmune
sepsis
sickle cell anemia
classic anemia Sx
elevated erythropoietin (EPO)
jaundice
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what is polycythemia vera? pathophysiology? manifestations?
excessive RBC production
JAK2 mutation increases EPO receptor sensitivity -> increased proliferation for RBC precursors regardless of EPO levels
increased viscosity + thrombocythemia -> hypercoagulable state + elevated BP = increased thrombosis risk
intense painful itching caused by water (aquagenic pruritus)
survival: 10-15 yrs possible but less than 18 months if untreated
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what are 5 things necessary for proper RBC development?
healthy bone marrow
erythropoietin -> stimulate RBC maturation
iron -> hemoglobin synthesis
vitamin B12 -> DNA synthesis
folic acid -> DNA synthesis
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what is the iron RDA for male, female and pregnant?
11mg/day
18mg/day
27mg/day
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what is iron necessary for?
Hb, myoglobin, some enzymes
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what are causes of iron deficiency?
blood volume expansion -> ex: pregnant, puberty
chronic blood loss (GI bleed, menses)
mostly increase in demand related
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what are consequences of iron deficiency?
iron deficiency anemia (severe -> tachycardia + angina
impaired myoglobin synthesis
impaired enzyme synthesis
impaired child development
impaired cognition
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what are diagnosis of iron deficiency?
hallmarks:
1- microcytic/hypochromic RBCs
2- absence of aggregated ferritin
lab tests to confirm:
decreased RBC + reticulocyte count
decreased Hb + hematocrit values
decreased serum iron content
increased serum iron-binding capacity
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what are indications of ferrous iron salts?
Tx + prevention of iron deficiency anemia
all formulations have equivalent efficacy + toxicity
ferrous sulfate = preferred choice because cheaper
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what are adverse effects of ferrous iron salts?
GI disturbances (dose dependant) -> nausea, diarrhea, heartburn
teeth staining
iron poisoning in children = leading poisoning fatality -> acidosis + shock, antidotes = gastric lavage or iron chelating agents
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what are drug interactions of ferrous iron salts?
antacids decrease absorption/ vitamin C increases absorption
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what are indications of iron dextran?
Px for whom oral iron is inefficient/impossible (GI disease)
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what is optimal dosage of oral iron salts? parenteral iron dextran?
65mg 3x/day for 200mg, best between meals to max absorption
depends on BW + severity of anemia
safety: fatal anaphylactic reactions -> test dose before full blown administration, IV has lower risk than IM, have epinephrine + reanimation equipment ready
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what are nursing assessments for iron deficiency?
determine cause
monitor reticulocyte + Hb for success/failure
avoid therapeutic combinations w/other iron, folic acid or B12 meds
duration: until Hb normal, usually 1-2 months
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what is main utility of B12?
folic acid activation
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what are causes of B12 deficiency?
impaired absorption
insufficient diet (very rare)
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what are diagnosis of B12 deficiency?
megaloblast detection
plasma B12 levels
B12 absorption test
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what are consequences of B12 deficiency?
megaloblastic (pernicious) anemia -> severe Sx = systemic hypoxia, heart failure + dysrhythmias
nervous system injuries -> demyelination of neurons, long term deficiency (permanent damage), unrelated to folic acid or DNA
GI disturbances
decreased WBC + platelets
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what is cyanocobalamin?
drug of choice for all B12 deficiencies
may produce hypokalemia -> RBC synthesis requires K+
oral: mild/moderate deficiency pt, even if lack of intrinsic factor as around 1% is still absorbed
parenteral (NEVER IV): best for pt w/severe deficiency
intranasal: good alternative to parenteral
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what are nursing guidelines for moderate B12 deficiency?
only manifestation = megaloblasts
no neuronal damage or WBC decrease
manage via B12 supplements
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what are nursing guidelines for severe B12 deficiency?
Sx: disruptions of all blood cells + GI + neuronal injuries
treatment: IM B12 + folic acid injection + blood replacement + platelet transfusion
monitor success: megaloblast disappear + reticulocyte count increases
potential neurologic recovery is inversely proportional to duration of deficiency
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what are nursing guidelines for long term therapy for B12 deficiency?
pt with B12 malabsorption -> intrinsic factor deficiency
monthly injections or very large oral doses
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what is folic acid necessary for?
DNA synthesis
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can folic acid deficiency develop rapidly?
yes due to daily loss
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what are causes of folic acid deficiency?
alcoholism: poor diet, enterohepatic folate recycling decreases
sprue: malabsorption syndrome
very rare: drug induced
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what are consequences of folic acid deficiency?
similar to B12 except NO neuronal injuries
potential increase of colorectal cancer + atherosclerosis
neural tube defect
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what are diagnosis of folic acid deficiency?
megaloblastic anemia with high B12 levels
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what is folic acid supplement? indications? adverse effects?
pteroylglutamic acid: inactive folic acid -> rapidly activated upon absorption
already active form are no more effective + more expensive
folic acid deficiency Tx + prophylaxis
initial severe B12 megaloblastic anemia therapy
non short term
increased colorectal cancer + atherosclerosis risks long term
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what are nursing guidelines for folic acid deficiency?
intervention should match cause: poor diet -> not supplements
malabsorption -> folate supplements
prophylactic supplements: only for soon to be pregnant, pregnant, lactating
severe deficiency management:
initially IM injection of B12 + folic acid -> combination fastens recovery
continue w/oral folate only
monitor success same as B12
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what is leukocytosis?
increased leukocyte count -> normal protective response
however abnormal when count doesn't go back down or rises for no reason -> pathologic condition or myeloproliferative disorders
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what is leukopenia?
decreased leukocyte count < 4000 cells/mm3
decrease in neutrophils
caused by bone marrow suppression
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what is neutropenia? what are causes?
neutrophils < 2000/ uL
inversely proportional to infection risk
bone marrow suppression (ex: chemo, anemia, anorexia)
increased destruction
severe prolonged infection
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what is agranulocytosis?
neutrophil count is 0
complete bone marrow destruction or drug induced
leads to recurrent and persistent life-threatening infection
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what is infectious mononucleosis? what are manifestations?
acute viral/bacterial infection of B cells
85% of cases -> Epstein Barr virus (HSV-4)
more than 90% of adults have indications of subclinical EBV infections
called kissing disease
self limiting, recovery within weeks
sore throat + fever
complications in immunocompromised (HIV!!)
splenic rupture is rare (0.1-0.5%)
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what are leukemias?
uncontrolled proliferation of malignant leukocytes -> overcrowding of bone marrow -> decreased production + function of normal hematopoietic cells
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what are leukemias classified based on?
1- progenitor cells -> myeloid or lymphoid
2- rate of proliferation -> acute vs chronic
ALL - acute lymphoid leukaemia
AML -acute myeloid leukaemia
CLL - chronic lymphoid leukemia
CML - chronic myeloid leukemia
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what is pathophysiology of acute leukemias? manifestations?
characterized by undifferentiated or immature cells, usually a blast cell
onset of disease is abrupt and rapid
strong genetic ethology w/chromosomal abnormalities
similar across variety of acute leukemias
fatigue (anemia), bleeding (thrombocytopenia), fever (infection)
splenomegaly + hepatomegaly
pain in bones + joints
CNS - leukaemia infiltration or cerebral bleeding
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what is pathophysiology of chronic leukemias? manifestations?
cells are more differentiated but do not function normally
relatively slow progression
CLL involved mostly B cells/ CML chimeric BRC-ABL fusion
CLL: 70% asymptomatic at time of dx, lymphadenopathy + suppression of humoral immunity
CML: splenomegaly + hyperuricemia (gout arthritis), terminal blast phase (rapid progressive leukocytosis survival 3-6 months)
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what are malignant lymphomas?
diverse group of neoplasms (cancer) that develop from proliferation of malignant lymphocytes in lymphoid system
often originates from lymph nodes/lymphoid tissue of stomach or intestines
result of genetic mutations or viral infection
produce tumor masses
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what is pathophysiology of Hodgkin lymphoma (HL)?
unknown malignant transformation trigger
Reed-Sternberg (RS) cells: large binucleate malignant transformed lymphocytes -> hallmark of HL
hypothesis: abnormal B cell survives thanks to EBV infection -> leads to first few RS cells -> RS cells release cytokines -> inflammatory response
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what are manifestations of Hodgkin lymphoma (HL)?
progression from lymph nodes to lymph nodes
pressure + obstruction of local lymph nodes
development of systemic symptoms of infection (fever, fatigue...)
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what is pathophysiology of Non-Hodgkin lymphoma (NHL)?
acquired mutation -> cell immortalization + proliferation -> progressive expansion of B cells (80-90%), T cells or NK cells
NO RS cells
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what are manifestations of Non-Hodgkin lymphoma (NHL)?
similar to HL
begin as lymphadenopathy (enlarged lymph node)
entranodal involvement = main difference
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what is pathophysiology of Burkitt lymphoma ?
fast growing B cell tumor
EBV-induced mutations in c-Myc Proto-oncogene
Boosted by immunocompromised status (HIV!!)
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what are clinical features of Burkitt lymphoma ?
tumor primarily in the jaw and facial bones
extra nodal locations develop over time
highest prevalence in Africa in New Guinea
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what is pathophysiology of multiple myeloma ?
masses grow slowly in + destroy bone marrow -> leads to pancytopenia (low RBC, WBC + platelets)
multiple genetic mutations + translocation
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what is manifestations of multiple myeloma ?
plasma cells produce defective antibodies (M protein) -> proteinuria (90%) + hyper viscosity syndrome (20%)
secret growth factors + bone resorption-promoting factors -> bone lesions + hypercalcemia
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what are hematopoietic growth factors (HGF)? what are therapeutic applications?
naturally occurring hormones regulating proliferation + differentiation of blood cells also called colony stimulating factors (CSF)
1-post chemo platelet + WBC regeneration
2- boost bone marrow transplant recovery
3- boost RBC synthesis in chronic renal failure (CRF) patients
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what are characteristics of erythropoietin?
primary function -> stimulate maturation of RBCs
other functions: angiogenesis modulation, cell injury apoptosis inhibition -> maintain cellular integrity
alternative to blood transfusions
inefficient w/out B12 + folate + iron
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what is purpose of Epoetin Alfa (EPO)? admin? indications? adverse effects?
decrease transfusion requirements
parenteral injections only
1- anemia in chronic renal failure (CRF) Pt: no QOL improvement
2- chemo-induced anemias: palliative purpose only
3- elevate RBC count prior to surgery: only if anticipate hemorrhage
4- AIDS pt on zidovudine
1- hypertension in CRF pt, due to increase in hematocrit
2- serious cardiovascular events: heart failure, stroke, MI, cardiac arrest
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what is purpose of Darbepoetin Alfa? admin?
it is a long acting EPO (longer T1/2), same toxicity + efficacy profile as epoetin alfa
purpose: decrease # of injections
1- anemia associated w/CRF
2- anemia associate w/chemo
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what are nursing EPO considerations?
safety -> minimize serious cardio events in CRF Pt
dosage should be lowest effective RBC elevation
decrease dosage if Hb increase > 1g/dl in 2wks
temporarily hold Tx if Hb > 11g/dL
warnings -> contraindicated for all cancer pt on curative/remission
must be combined w/anticoagulant for preoperative RBC elevation
monitor
- measure Hb levels at baseline + 2x/week until target
- blood them, iron, complete blood count routinely
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what are leukopoietic growth factors (LGF)? what are 2 agents?
stimulate WBC (leukocytes) production
filgrastim (G-CSF, for neutrophils)
sargramostim (GM-CSF, for neutrophils + macrophages)
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what is physiology of LGFs? therapeutic use? administration? adverse effects?
released in response to inflammation
increase neutrophil synthesis + macrophages
decrease neutropenia during chemo + bone marrow transplant (BMT) pt
increase pre-BMT hematopoietic stem cell (HSC) harvest
accelerate BMT recovery + increased failed BMT survival time
increase neutrophil recovery + decreased infections in acute myeloid leukaemia pt
parenteral administration only
short term: none + no interactions
bone pain: usually mild, use NSAIDs
leukocytosis/thrombocytosis: only with high dosage
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what are thrombopoietic growth factors (TGF)? what is main one?
stimulate platelet (thrombocytes) production
oprelvekin = endogenous interlukin-11
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what is action of TGFs? therapeutic use? administration? adverse effects?
stimulates HSC differentiation into megakaryocytes
decreased thrombocytopenia + platelet transfusion during chemo
efficacy decrease in highly myelosupressive chemo regimen
parenteral only
increase kidney sodium/water retention -> edema + increase plasma V
tachycardia + AF -> possibly related to plasma V
rare case: severe anaphylactic
