Chapter 13 Flashcards
2 types of hemoglobin
adult (HbA)- adults and children > 6 months
fetal (HbF)- 3-9 months in fetus
Adult hemoglobin (HbA)
2 alpha chains
2 beta chains
adults and children > 6 months old
Fetal Hemoglobin
2 alpha chains
2 GAMMA chains
have ^ O2 affinity, takes from mom
predominant 3-9 months gestation
how many molecules of O2 can be carried by one hemoglobin?
4
How much hemoglobin we make, depends on how much ___ we have
iron
65% of iron can be found in the ___
hemoglobin (circulating)
15-30% of iron can be found stored in the ___
liver
&
reticuloendothelial cells of BONE MARROW
“where RBC’s go to die”
spleen
what happens when a RBC is destroyed in the spleen?
the iron from the hemoglobin is released into the circulation and returned to the bone marrow, liver or other tissues
iron is typically derived from ___ and absorbed in the ___ .
meat small intestine (duodenum)
an incomplete, non function protein that turns into transferin when iron gets absorbed into duodenum.
Apotransferin
refers to the production of RBC
Erythropoeitin
erythropoesis is stimulated by?
low O2
Erythropoeitin is made where and stimulated into action where?
kidney
Bone marrow- stimulated stem cells to create hemocytoblasts –> proerythroblasts
RBC’s last about ____
120 days
why do RBC’s only last a short amount of time? (2)
- metabolic activity decreased with age (creates weakened cell membranes)
- eventually, RBC’s break as they squeeze through spleen capillaries (don’t have strong cell membrane)
If old and Damaged, RBC’s get phagocytized by cells from the:
liver, spleen, bone marrow, lymph nodes
when RBCs are destroyed, the heme…
gets converted into bilirubin (bile pigment)
when rbc’s die, ____ takes the___to the liver where its stored as Ferin.
transferin, iron
Bilirubin is removed from the blood as ____ or _____.
conjucated bilirubin
or
unconjucated bilirubin
_____ attaches to glucocorinide which makes it soluble in plasma and can be excreted in bile.
Conjugated bilirubin
___ is plasma insoluble. and free floats in the blood causing a yellow pigment to the skin called ___
unconjugated bilirubin
jaundice
free hemoglobin in the blood is called:
hemoglobinemia
too much hemoglobin being excreted in the urine is called ___
hemoglobinuria
defined at the abnormally low # of circulating RBC’s or hemoglobin
Anemia
manifestations of anemia depend on:
- severity
- rapidity of its development
- underlying pathological mechanisms
- persons age and health status
symptoms of anemia:
pallor- skin, mucous membranes, conjuctivate, nail beds
tachycardia &Palpitations- heart compensating for low O2
premature death of RBC’s in bone marrow is called:
Hematopoiesis
RBC count and hemoglobin provide info on:
while
RBC size, shape and color provide info on:
Severity of anemia
Cause of anemia
Blood loss anemia can be: ___ or ___
acute or chronic
acute blood loss affects:
while
chronic blood loss affects:
Intravascular volume: create cardiovascular shock or collapse
DOESN’T affect blood volume: leads to iron deficiency with depleted stores (GI bleed, menstrual)
Disease characterized by premature destruction of RBC’s in blood circulation
hemolytic anemia
hemolytic anemia triggers:
^ in Erythropoeiten production which creates an ^ # of reticulocytes floating in the blood.
hemolytic anemia is characterized into two categories:
- Intrinsic- hereditary, something is wrong with inside of RBC
- Extrinsic- acquired factors like immune dysfunction, mechanical trauma, infections.
In hemolytic anemia, the hemoglobin binds to __ and ___ to be carried back to liver.
haptoglobin
and
albumin
RBC’s are made in the ___ and destroyed in the ___
bone marrow, spleen
3 main causes of anemia:
1- blood loss
2- Hemolysis
3- Impaired RBC production
Common types of anemia (3)
1- Iron deficiency- no hemoglobin present (pale cells)
2. Megaloblastic- DNA errors so cell gets too big
3- Sickle cell- gene mutation that causes change in structure ( collapse when O2 is not bound to them)
3 common types of Hemolytic Anemia
1- membrane disorders (hereditary spherocytosis, acquired hemolytic newborn disease)
- Hemoglobinopathies- sick cell, thalassemia (treat by taking spleen out)
- G6PD deficiency
Sickle Cell Anemia
inherited disorder where abnormal hemoglobin (HbS) leads to chronic hemolytic anemia, pain, and organ failure
HbS gene-
transmitted by recessive inheritance. (heterozygote with 1 HbS gene) OR sickle cell disease (homozygous 2 HbS)
HbS gene is caused by
mutation in B chain hemoglobin molecule
2 major consequences or RBC sickling
1- Chronic Hemolytic Anemia- bc of sick structure, RBC’s only live for about 20 days
2.- Blood Vessel Occlusion- disrupts blood flow causing ischemia and pain.
Factors associated with sickling and vessel occlusion:
- Dehydration- ^ hemoglobin concentration, attributes to polymerization and resultant sickling
- Acidosis- reduces affinity of hemoglobin to O2= more deoxygenated hemoglobin= sickling
- Hypoxia
Symptoms of Sickle Cell Anemia
(1-2. caused by sickled cell death)
(3-5 caused by sickled cells blocking capillaries)
- Hyperbilirubinemia- breakdown of hemoglobin –> jaundice
- Pigment gallstones
- Prone to Infections- low blood flow, O2, congestion causes Asplenia
- Vaso-occlusive pain crisis
- Acute Chest Syndrome
Vaso-occlusive pain crisis
Acute Chest Syndrome
- common in abdomen, chest, bones, and joint. caused by ischemia to those areas.
- atypical pneumonia resulting from pulmonary infarction. #1 cause of death in persons with sickle cell. * shortness of breath, fever, chest pain, cough.
If your spleen is affected due to sickle cell, you are more common to get ___
infections.
more susceptible to certain bacterias, especially babies and kids!
Sickle Cell Treatment (kids)
- Penicillin, updated vaccinations, specific pneumococcal vaccine
- Hydroxyuria- drug that inhibits DNA synthesis and can help prevent some complications
Treatment for intense cases of sickle cell:
- bone marrow transplant
(at risk for graft vs. host disease) bone marrow attacks the persons body bc they don’t have the same cell surface molecules that they’re used to.
Hemolytic Anemia- Hemoglobinopathies
- sickle cell disease & thalassemia
Thalassemia (different synthesis of 1 of the 2 chains)- deficiency of hemoglobin bc of this.
beta- more serious (Mediterranean)
alpha- less serious
microsystic
hypocromic
small size
pale or low color
Thalassemia (alpha)
- defective gene for alpha chain synthesis
- 1-4 defective genes
- Affects both fetal and adult Hb
- In fetus: gamma4 Hb may form
- In adult: beta4 Hb may form
- uneven hemoglobin synthesis
Thalassemia (beta)
*more severe form- transfusion dependent. have to have transfusions or you cant carry O2.
- > 100 defective gene mutations
- Affects only adult Hb
- Alpha4 Hb may form
too much of alpha chain causes insoluble aggregation of RBC’s. this is called:
Heinz Bodies
Hemolytic Anemia- G6PD deficiency
- defective glucose 6 dihydrodrinase.
- Defect in pathway that makes G6PD. which makes blood cells more susceptible to oxidative damage.
- Causes damage to plasma membrane of cells- they burst!
- Genetic disorder that is common in Males (X linked)
- No treatment for it- DON’T TAKE Oxidative Drugs
Anemias from Deficient RBC Production
- iron Deficiency Anemia
- Megaloblastic Anemia
- Aplastic Anemia
- Chronic Disease Anemias
Iron Deficiency Anemia
Cause: blood loss(GI bleed, peptic ulcer) or deficient diet
- low hemoglobin and hematocrit
- low serum iron and ferritin
- Hypochromic and microcytic erythrocytes
- Poikilocytosis (irregular shape)
- Anisocytosis (irregular size)
Iron Deficiency Anemia Symptoms;
tired, out of breath, rapid HR, changes in color (waxy/pale), hair and nails are brittle, PIKA or neurological issues
Megaloblastic Anemia
also called Pernicious Anemia
Causes: lack of Vitamin B12 and Folic Acid
- Impaired DNA synthesis –> enlarged RBC’s
- Erythrocytes are large, often with oval shape
Vitamin B12
Needed for DNA synthesis and normal RBC maturation and division.
- absorbed from animal poteins in mucosal lining of stomach.
- when cells in stomach are damaged and can’t absorb B12: Atrophic Gastritis
Folic Acid
Needed for DNA synthesis and maturation of RBC. the Cells get too big to be absorbed through GI tract.
* most common dietary deficiency
- Causes: celiac disease, antipileptic disease.
which type of deficiency is caused by pernicious anemia?
Vitamin B12
Relative Polycythemia-
Absolute Polcythemia-
- loss of plasma volume (dehydration)
- increased RBC mass ( ^ in # of RBC’s)
- Primary- Neoplastic- everything is increased red, white, hemoglobin
- Secondary- ^ erythropoietin. (living at high altitudes, smoking, lung conditions)
Age-Related Changes in Neonate RBC’s
- red cells vary during first week, then drop (normal)
- HbF turns to HbA transition (around 6 months- alpha gamma to alpha beta switch)
- Anemia of prematurity- premi babies don’t have a lot of RBC’s to begin with. need to increase over time.
Hyperbilirubinemia in Neonate
- common cause of jaundice in neonate
- risk of Kernicterus
- Treatment with UV light- excites bilirubin turning it into a water soluble molecule to be removed.
Kernicterus
- untreated bilirubin leads to this in neonates*
- unconjugated bilirubin is fat soluble not water. It crosses the blood/brain barrier and accumulates in brain cells causing brain damage.
- lethargic, don’t feed well, behavior changes.
_ leads to seizures, death, or long term brain damage.
Hemolytic Disorders of the Newborn
- Erythroblastosis Fetalis-
- Rh+ babies born to Rh- mothers.
- Immune response to invader of 2nd baby only
- causes hemolysis or agglutination of babies RBC’s.
