anemia Flashcards
-2 ways to get Iron. Iron ingested or breakdown from older erythrocytes
-Iron travels to the stomach and HCL + Vit C changes it from Ferric 3+ to ferrous 2+
-Absorption of FE 2+ occurs in the duodenum and upper Jejunum by the enterocytes
-Fe 2 goes into enterocytes
-Next it goes through Ferroportin (door) changes back to F 3+ goes into blood stream bound to Transferrin
-Iron gets transported on Transferrin
1 Goes to Bone marrow to Makes RBC
2 Gets stored the the live as ferritin
-30-40mg of iron to make RBCs
-most is absorbed in GI -> transferrin -> stored in liver
-macrophages store iron
How to turn off Iron absorption
Hepcidin is a regulatorinhibator
1.Shut door on Ferroportin : close
2. Stops macrophages do not release Iron
iron tests
-Serum iron blood test -> how much iron inside body
-Ferritin test -> how much iron is stored inside cells
-TIBC test (total iron-binding capacity) -> all proteins needed to link iron including transferrin (means the total transferring available).
-UIBC test (unsaturated iron-binding capacity) -> how much iron for saturation of transferrin.
-Transferrin saturation- a percentage to tell how much transferrin is full with iron.
iron facts
-Morning iron is higher than the rest of the day’s iron levels by 30%.
-67% of the body’s iron is inside hemoglobin in red blood cells, other iron is bound to transferrin in blood or ferritin in bone marrow, or stored in more body tissues.
-3.5% of the total iron in the body is inside Muscles as myoglobin.
-27% of all iron inside cells are stored in Ferritin & hemosiderin
iron deficiency anemia labs
transferrin - protein that carries the iron
-TIBC- high
iron deficiency anemia
-MC type of anemia.
-body doesn’t have enough iron
-body needs iron to make hemoglobin
-body can’t get amount of oxygen it needs.
-many people don’t know they have iron-deficiency anemia -> possible to experience sx for years w/o knowing cause.
causes of iron deficiency anemia
-kids who drink a lot of milk and not hungry otherwise
-women and in people who have a diet that is low in iron
-highest risk:
-Women who menstruate, particularly if menstrual periods are heavy
-Women who are !pregnant or breastfeeding or those who have recently given birth!
-major surgery or physical trauma
-GI diseases such as celiac disease (sprue), inflammatory bowel diseases such as UC, or Crohn disease
-People with PUD
-bariatric procedures, especially gastric bypass operations
-Vegetarians, vegans, and other people whose diets do not include iron-rich foods (Iron from vegetables, even those that are iron-rich, is not absorbed as well as iron from meat, poultry, and fish.)
-Children who drink more than 16-24 ounces a day of cow’s milk (Cow’s milk not only contains little iron, but it can also decrease absorption of iron and irritate the intestinal lining causing chronic blood loss.)
less common causes of iron deficiency
-Blood loss from the gastrointestinal tract due to gastritis (inflammation of the stomach), esophagitis (inflammation of the esophagus), ulcers in the stomach or bowel, hemorrhoids, angiodysplasia (leaky blood vessels similar to varicose veins in the gastrointestinal tract), infections such as diverticulitis, or tumors in the esophagus, stomach, small bowel, or colon
-Blood loss from chronic nosebleeds
-Blood loss from the kidneys or bladder
-Frequent blood donations
-Intravascular hemolysis -> RBC break down in the bloodstream, releasing iron that is then lost in the urine. This sometimes occurs in people who engage in vigorous exercise!, particularly jogging. This can cause trauma to small blood vessels in the feet, so called “march hematuria.” Intravascular hemolysis can also be seen in other conditions including damaged heart valves or rare disorders such as thrombotic thrombocytopenia purpura (TTP) or diffuse intravascular hemolysis (DIC).
signs and symptoms of iron deficiency anemia
-fatigue
-pallor
-poor concentration
-presyncope/syncope
-weakness
-dyspnea
-cant get up the stairs
-smooth tongue
-craving ice
koilonychia
dx and tx of iron deficiency anemia
-iron sat is low
-normo -> micro -> micro hypochromic
stages in development of IDA
complications of IDA
-Mild usually doesn’t cause complications
-if left untreated -> can become severe and lead to health problems, including the following:
-Heart problems
-rapid or irregular heartbeat
-heart must pump more blood to compensate for the lack of oxygen carried in your blood when you’re anemic -> enlarged heart or heart failure
-Problems during pregnancy
-severe iron deficiency anemia has been linked to premature births and low birth weight babies
-it is preventable in pregnant women who receive iron supplements as part of their prenatal care
-Growth problems
-infants and children -> severe iron deficiency can lead to anemia as well as delayed growth and development
-associated with an increased susceptibility to infection
iron replacement therapy
-they should go to GI so you arnt missing a colon cancer
ferrous sulfate
-tx for iron deficiency anemia
-dose- 325mg tid, which provides 180mg of iron daily of which 10mg is usually absorbed
-pts who cant tolerate iron on empty stomach should take it with food
-PO
-give with stool softener
-iron causes constipation
lead poisoning
-usually made via screening program
-if suspected must obtain detailed history:
-onset of sx
-hx of PICA
-assess of potential sources
-family hx of lead poisoning
-PE:
-pallor and hyperactivity
-burton lines on gums
-decreased stature
-wrist drop, and cognitive dysfunction
-signs of elevated ICP:
-impaired consciousness
-bradycardia
-HTN
-respiratory depression
-papilledema
-coma
-basophilic stippling on the RBC on cytology -> diff from IDA
-old homes in the city, old pipes, water
-children absorb it more
mild cases of lead poisoning
-hypochromic microcytic anemia -> dont confuse it with iron deficiency
hereditary hemochromatosis
-Serum ferritin: Measures the amount of iron stored in the liver
-Serum iron: Measures the amount of iron in the blood after fasting
-Transferrin saturation: Measures the amount of iron bound to transferrin, a protein that carries iron in the blood
-LFTs: Check for liver inflammation and normal liver function
hemochromatosis triad
-set of symptoms that historically identified the condition, but it’s less common today due to earlier diagnosis:
-Hepatomegaly: An enlarged liver, which occurs in 95% of patients with symptoms
-Skin hyperpigmentation: Also known as bronze skin, this is the condition’s namesake
-Diabetes: A type of diabetes mellitus
genes for hemachromatosis
-C282Y
-every 3-4 months get blood removed
dx of hemochromatosis
-ferritin goes up with any inflammation
-look at the saturation
approach to macrocytic anemia
macrocytic with neutrophils >5 lobes
megaloblastic
macrocytic anemia: megaloblastic vs non-megaloblastic
causes of macrocytic megaloblastic anemia
-neurological symptoms for b12
vitamin b12 deficiency
-can be taken by mouth, shot, or inhaling through nose
-neurological symptoms!
-mimics elderly sx -> check them
-absorption of b12 in the terminal ileum
-bypass surgery is deficient
-you need intrinsic factor
-B12 transformed in the liver and stored
neurological changes in b12 deficiency!
folic acid deficiency anemia
low folate in pregnancy
-causes neural tube defects
-fetus needs folate
-can be low in the pregnancy -> you NEED IT
macrocytic megaloblastic anemia causes
-dont need to know all the drugs
-methotrexate for RA
macrocytic non-megaloblastic anemia causes
high MVC in heavy alcohol use
congenital hemolytic anemia
-Genetic conditions of RBC Membrane
-Hereditary spherocytosis
-Hereditary elliptocytosis
-Genetic conditions of RBC metabolism (enzyme defects). This group is sometimes called congenital nonspherocytic (hemolytic) anemia, which is a term for a congenital hemolytic anemia without spherocytosis, and usually excluding hemoglobin abnormalities as well, but rather encompassing defects of glycolysis in the erythrocyte.[2]
-Glucose-6-phosphate dehydrogenase deficiency (G6PD or favism)
-Pyruvate kinase deficiency
-Aldolase A deficiency
-Hemoglobinopathies[3]/genetic conditions of hemoglobin
-Sickle cell anemia
-Congenital dyserythropoietic anemia
-Thalassemia
hemolytic anemia labs
-reticulocytes HIGH
-MCV HIGH
-bilirubin very HIGH
-haptoglobin LOW
-plasma hemoglobin HIGH
-urinary hemoglobin N or HIGH
-LDH HIGH
symptoms of hereditary spherocytosis
-inherited blood disorder of RBC
-RBC are round rather than donut shape
-destroyed in the spleen at increased rate bc of abnormal shape
-anemia, jaundice, enlarged spleen
-symptoms of anemia- pale skin (pallor) compared to your child’s normal colour, low energy (fatigue), irritability
-lifelong condition
-can be managed and treated
-some benefit from having a portion or all of spleen removed
-discuss options with doctor
red blood cell membrane structure: spherocytosis
-problem with ankyrin and spectrin
hereditary spherocytosis labs
MCHC
dx of spherocytosis
-osmotic fragility! (PANCE)
osmotic fragility
test of choice for spherocytosis
-Osmotic fragility is a blood test to detect whether red blood cells are more likely to break down.
spherocytosis with viral illnesses: aplastic crisis
-does not usually affect your child’s blood counts
-BUT in some viral illnesses can worsen anemia and increase jaundice
-This condition is called an aplastic crisis
-Parvovirus is a common virus associated with an aplastic crisis in HS and may cause the following symptoms:
-Significant change in pallor or jaundice
-Lethargy (lack of energy)
-New onset headache (along with other symptoms listed here)
-Light-headed or dizzy
-Shortness of breath with activity
spherocytosis tx
-Phototherapy
-Jaundice can be a problem in the newborn period if the bilirubin levels are too high and may require treatment, such as phototherapy. Phototherapy involves exposing the skin to a special blue light that changes bilirubin to a form that can be passed out of the body more easily.
-Blood transfusion
-In some cases, a blood transfusion is required for severe anemia. This is most common in the first year of life but may be ongoing if the anemia is severe. Most children with HS do not need blood transfusions on a regular basis.
-Splenectomy- this treats
-Cholecystectomy- might need to get it out bc of gallstones -> doesnt treat
hereditary elliptocytosis labs
hereditary elliptocytosis
due to various defects in skeletal proteins, spectrin and protein 4.1
-results increased membrane rigidity and decreased cellular deformability
-autosomal-dominant mode of inheritance
-elliptocytes varies from 50-90%
-osmotic fragility normal and increase (not as helpful)
-tx- transfusions, splenectomy, prophylactic folic acid
folic acid
-1mg daily
-HE and HS tx
G6PD deficiency
-africa
-south america
types of G6PD deficiency
severe*
-most of the time they have intermittent hemolysis
function of G6PD
G6PD risk factors
-legumes
-infections
-certain chemicals
drugs that trigger G6PD hemolysis
-henna
labs found with G6PD
-severe anemia with Hb 4-6g/dl PCV and RBC count are decreased
-WBC increased in many cases
blood film of a H6PD deficient pt with acute hemolytic anemia
thalassemia
-worlds MC genetic disease
-autosomal recessive inheritance
-millions of carriers and 100s of 1000s of pts worldwide
thalassemia: a quantitative defect
world incidence of alpha thalassemia
world incidence of beta-thalassemia
alpha-thalassemia
-4 is normal number of alpha-globin genes
-if 1 or 2 genes are missing- alpha thalassemia trait, causing mild microcytosis with or without anemia
-if 3 genes are missing- alpha thalassemia (HbH disease)
alpha thalassemia
alpha thalassemia dx
-HbH inclusion bodies
-severe hypochromia
-results in an excess of beta globins, which leads to the formation of beta globin tetramers (β4) called hemoglobin H. These tetramers are more stable and soluble, but under special circumstances can lead to hemolysis, generally shortening the life span of the red cell.
beta-thalassemia
-chromosome 11 problem
beta thalassemia: thalassemia minor
electrophoresis testt
-hemoglobin A2 is how you dx -> HIGH
beta thalassemia: thalassemia major
peripheral blood smear: beta thalassemia
thalassemia pathophysiology
clinical features of thalassemia
-RBC is high
-microcytic anemia
skeletal changes: thalassemia
thalassemia tx
Blood transfusions: You may need frequent blood transfusions (as much as every two weeks) with beta thalassemia major. During the procedure, you receive blood from a donor. The influx of blood from a transfusion supplies red blood cells needed to carry oxygen to tissues throughout your body.
Iron chelation therapy: Iron is an important part of the hemoglobin protein allowing it to carry oxygen. Too much iron, however, can be harmful. Iron chelation therapy can help prevent iron overload.
Folic acid supplements: Folic acid can help boost your body’s ability to make red blood cells. Your doctor may recommend supplements if you have beta thalassemia minor. You may also take folic acid in addition to receiving regular blood transfusions if your condition is more severe.
Luspatercept: If you have severe thalassemia, you may receive an injection (shot) of luspatercept every three weeks to help your body make more red blood cells. Luspatercept improves anemia in people diagnosed with beta thalassemia who are receiving blood transfusions.
Bone marrow and stem cell transplant: You may receive bone marrow stem cells from a donor. Bone marrow stem cells eventually mature into red blood cells. Replacing your bone marrow stem cells with the stem cells of a healthy donor can cure beta thalassemia. Unfortunately, finding a compatible donor can be a challenge. Also, this type of transplant is considered a high-risk procedure.
thalassemia is preventable
in cyprus and in Greece, extensive screening
-public health education have led to a nearly complete disappearance of thalassemic births
sickle cell anemia: RBC membrane changes
-shape of it causes it be be clogged -> pain
sickle cell: gene
how is sickle cell caused
glutamine changes to valine
sickle cell anemia: sickling episodes
-infection
-dehydration
-hypoxia
-renal circulation (mild)
sickle cell anemia morbidity
-MCC of morbidity is vaso-occlusion
-reticulocytes and sickled cells adhere to endothelium of small and large vessels, leading to tissue infarction and organ damage
sickle cell anemia: organs involved
-MC:
-skin (ulcers)
-bones (painful crises, infections)
-respiratory system (acute chest syndrome)
-brain (infarction)
-eyes (retinopathy)
sickle cell anemia: dx
-peripheral smear
-reticulocyte count
-high MCV (secondary folic acid deficiency is common)
-Hb electrophoresis
-howell-jolly body
clinical features of sickle
sickle cell anemia crisis tx
-hydration
-analgesia
-steroids
-antibiotics
-ventilatory support
-do NOT give transfusions -> hyperviscosity
sickle cell anemia: exchange transfusion
sickle cell anemia: chronic tx
hydroxyurea
-new mainstay of tx
-for life
-know this
-immunosuppressant
-takes 6 months to work
-change it very slowly
transfusion: sickle cell
advantages and disadvantages of simple vs exchange transfusions
clinical presentation of sickle cell
hemoglobin c
2-Hb C disease
-target cells
-hemoglobin C crystals
hemolytic anemias
classification of hemolysis
intra vs extravascular hemolysis
acquired hemolytic anemia: immune vs non immune
lab findings in hemolysis
schistocytes
extravascular vs intravascular hemolysis
-symptoms are about the same
-extravascular- enlarged spleen
warm auotimmune hemolytic anemia causes
-antiviotics optimally active at 37 degrees
-IgG
-causes:
-idiopathic
-secondary:
-SLE
-CLL
-lymphomas
-drugs- methyl dopa
warm autoimmune hemolytic anemia pathogenesis
warm autoimmune hemolytic anemia clinical features and investigations
-schistocytes
-helmet cells
direct coombs test
+
-warm autoimmune hemolytic
-sphyllis
-mono
warm autoimmune hemolytic anemia treatment
-corticosteroids
-splenectomy
-immunosuppresive - Azathioprine
-folic acid
-tx underlying cause
cold agglutinin disease
cold autoimmune hemolytic anemia investigations
cold autoimmune hemolytic anemia clinical features
cold autoimmune hemolytic anemia tx
autoimmune hemolytic anemia
look at the antibody
pre splenectomy / post splenectomy
-PRE SPLENECTOMY
-pneumococcal vaccination, both types of meningococcal (MenACWY and MenB) vaccination, and Hib vaccination should all be completed at least 2 weeks before a scheduled splenectomy!!!!!!!!!!!!!!
-POST SPLENECTOMY
-Patients who have had a splenectomy following trauma should continue antibiotic prophylaxis for a minimum of two years after surgery
-Howell–Jolly bodies!! are found in patients who have had splenectomies or are hyposplenic (e.g., sickle cell anemia) and rarely in megaloblastic anemias
-spherocytosis
-warm antibody
-sickle cell (autorejects)
paroxysmal cold hemoglobinuria
hemolytic anemia resulting from infections with microorganisms
babesia
-tics
-hemolytic
-flu like
what dx increases likelihood of IDA
PUD
-MCV much lower -> really small
-thalassemia
-follow up with electrophoresis
iron deficiency
A 14 year-old female has sickle cell anemia. Which factors below can increase the patient’s risk for developing sickle cell crisis? Select all that apply:*
a. Shellfish
B. Infection!!
C. Dehydration!! -> more get stuck
D. Hypoxia!!
E. Low altitudes -> it would be high
F. Hemorrhage!!
G. Strenuous exercise!!- stress in body
You’re providing education to a patient with sickle cell anemia who is taking Hydroxyurea. You will make it priority to tell the patient to?*
a. Consume foods high in calcium and potassium
B. Avoid sick people and maintain strict hand hygiene
C. Take this medication with at least 8 oz of water
D. Monitor your blood glucose level daily
A mother brings in her 8 month-old child to the ER. The mother reports the baby has recently started being extremely fussy, has a fever, and swelling in the hands and feet. The child is diagnosed with sickle cell disease. As the nurse you know that the swelling in the hands and feet in the infant is termed?*
A. Dactylitis!!!!
B. Erythromelaglia
C. Dyshidrotia
D. Phalitis
What is the primary risk to thalassemia major patients
who receive frequent and multiple blood transfusions?
A. Iron overload!!!!
B. Citrate toxicity
C. Polycythemia
D. Hyperviscosity
Hemoglobin H!!! disease is characterized by the deletion of………………………………….?
a) One alpha gene
b) Two alpha gene
c) Three alpha gene !!!!!
d) Four alpha gene- do not live
