Hema disorders Flashcards
What is anemia
disorder characterized by a reduction in total RBC and/or a decrease in hemaglobin
How is anemia caused
- Impaired RBC production
- excessive blood loss
- increased RBC destruction
Any combination of the three
Normal RBC number
men: 4.7-6.1 mcL
Women 4.5-5.2 mcL
RBC number counts
number of erythrocytes in 1 cubic mm of whole blood
Hb normal count
men: 13.5-17.5
Women: 12.0-15.5 g/dL
Hb is what
the o2 carrying pigment of red cells
Hct normal number
Men: 42045%
Women 37-48%
What are reticulocytes
immature RBC
what does reticulocytes tell us
bone marrow function
Normal reticulocyte count
in adults approx 3%
Mean cell volume
measures the average size of the rbc
Normal mean cell volume
80-100 fL
Mean Corpuscular hemoglobin (MCH)
average weight of hb per red cell
what is the normal MCH
27-33pg
Mean corpuscular hemoglobin concentration (MCHC)
average concentration of hemoglobin per erythrocyte
normal MCHC
32-36%
red cell distribution width (RDW)
a quantitate estimate of the uniformity of individual cell size
RDW normal
11.5-14.5%
What are the 3 categories of anemia based on the average size of RBCs?
- Microcytic
- macrocytic
- normocytic
Microcytic anemia’s MCV
<80 meaning that they are SMALL
macrocytic anemia’s MCV
> 100 meaning that they are LARGE
normocytic anemia’s MCV
80-99 meaning that they are normal size
3 classifications of anemia based on color
- hypochromic
- hyperchromic
- normochromic
Hypochromic anemia
RBC with less hemoglobin than normal
MCHC in hyopchromic anemia
low- appear pale in color
Hyperchormic anemia
RBC with more hemoglobin than normal
MCHC in hyperchromic anemia
high- appear dark or more red
Normochromic anemia
RBC apear neither pale nor dark
Microcytic anemia types:
- iron deficiency
- sideroblastic
- thalassemia
- anemia of chronic disease
Normocytic anemia types:
- anemia of inflammation and chronic disease
- hereditary spherocytosis
- G6PD deficiency
- Paroxysmal nocturnal hemoglobinuria
Macrocytic anemia types:
- B12 deficiency (pernicious anemia)
- Folate deficiency
Because of decreased tissue oxygenation, what symptoms manifest
- severe fatigue
- pallor
- weakness
- dyspnea
- dizziness
Because of the RBC level being low, what happens due to the subsequent decrease in blood volume
activation of the renin-angiotensin-aldosterone system promoting fluid retention and movement of interstitial fluid into the capillaries further diluting the plasma.
END RESULT: tachy or hf
Iron deficiency anemia types
microcytic-hypochromic
Most common type of anemia
iron deficiency anemia
most common reason for iron deficiency aneia
insufficient amount of iron intake/availability
Causes of IDA
- inadequate intake
- chronic or occult bleeding
- decreased ability to use Fe for heme synthesis
What would cause decreased ability to use Fe for heme
transferrin deficiencies and mitochrondrial defects
causes of chronic or occult bleeding
hemorrhage colitis cirrhosis GI ulcers esophageal lesions menorrhagia
what are the two categories of macrocytic anemias
- Megaloblastic
- non-megaloblastic
causes of megaloblastic macrocytic anemias
- folate deficiency
- B12 deficiency
causes of non-megaloblastic macrocytic anemias
- liver disease
- myelodysplastic syndrome
- increased reticulocyte count (Hemorrhage)
Why are folate and b12 important
required for cell DNA synthesis
deficiency in either results in impaired DNA replication of the RBC causing them to continue to increase in size
Clinical finding in macrocytic anemias
- fatigue
- dyspnea
- loss of appetite or weight
- diarrhea
- pallor
what is B12 deficiency also called
pernicious anemia
What causes pernicious anemias
autoimmune destruction of the gastric parietal cells which decreases the secretion of intrinsic factor
The intrinsic factor binds to B12 in the stomach and travels through the small intestine
when the complex reaches the ileum, it breaks down and B12 is absorbed
what are additional causes of pernicious anemias
- insufficient dietary intake of b12
- gastritis
- H. Pylori
- advanced age
- gastrectomy prosedures
dietary sources of b12
- liver
- beef
- chicken
- pork
- caption
- whole eggs
- dairy products
Excessive alchohol abuse can lead to
filate deficiency
Causes of folate deficiency
- alcohol abuse
- Medication interactions (anticonvulsants_
- malnutrition
Symptoms of folate deficiency
similar to b12 but without neurological symptoms
What does a folate deficiency do to RBC production
leads to abnormal RBC formation and premature RBC death
Folic acid rich foods
green-leafy veggies citrus beans rice/cereal fortified foods
??Two types of normocytic anemias
- hemolytic anemia
- blood loss anemia
Hemolytic anemia
“lysis of red blood cells”
causes of hemolytic anemia
These cause premature destruction or lysis or RBC:
- Infection: parasitic, heminthic, hemolytic toxin-producing bacterias, e. choli
- transfusion reaction
- autoimmune reactions: congenital or idiopathic
- drug-induced
When RBC’s lost due to bleeding faster than them being replaced
Blood loss anemia (can be acute or chronic)
chronic Blood loss anemia can result in
iron-deficiency anemia due to the depletion of iron stores
Aplastic anemia reticulocyte level
low
reticulocyte level in hemolytic and blood loss anemia
high
Aplastic anemia
Bone marrow failure
What causes the bone marrow failure in aplastic anemia
- Chemical or radiation exposure
- Viral induced: Hepatitis, Epstein-barr virus, cytomegalovirus
- antibiotics and other medications: PCN, chloramphenicol, phenytoin, diuretics, anti-diabetic drugs
- congenital defects: fanconi’s anemia
- tumors- Multiple myeloma
hemoglobinopathies
inherited disorders of erythrocytes
How many genes are involved in encoding synthesis of the alpha protein change for Hb
four
Where are the four encoding genes located for alpha Hb
chromosome number 16
How many genes are involved in encoding synthesis of beta protein chains for Hb
two
where are the genes located for the beta protein
chromosome 11
What are the two most common hemoglovinopathies
- Sickle-cell anemia
- Thalassemia
How does one get sickle cell
it is a inherited autosomal recessive disorder, both parents must given the recessive gene
Sickle cell is characterized by what
the formation of elongated “sickles” Hb molecules (HbS) which does not bind to oxygen well
What increase sickling tendencies of Hb in sickle cell
oxidative stress anxiety cold fever dehydration
What happens after only 10-15 days in sickle cell patients
lysis of the distorted RBC’s putting the person at risk for circulatory iron overload
The sickled RBC can also block cerebral, splnic and glomerular blood vessels causing the patient to be at risk for
strokes and splenic and kidney damage
Thalassemia (s) is caused by what
are a group of related inherited autosomal recessive disorders
person must inhearet a recessive from both parents
How is thalassemia different from sickle cell
thalassemia is characterized by many different genetic mutations causing a singel or multiple amino acid changes on alpha or beta chains causing varying degrees of distortion and dysfunction of rbc`
Lab values for pernicious anemia: MCV MCHC Reticulocyte count Ferritin Folate B12
MCV: high MCHC: normal Reticulocyte count: low Ferritin: normal Folate: normal or low B12: low
Lab values for folate deficient anemia MCV MCHC Reticulocyte count Ferritin Folate B12
MCV: high MCHC:normal Reticulocyte count: low Ferritin: normal Folate: low B12: normal or low
Microcytic Hypochromic anemias (3)
- Iron Deficiency
- Sideroblastic
- Thalassemia
microcytic normochromic anemia (1)
anemia of inflammation and chronic diseases
Microcytic Hyperchromic anemia (1)
hereditary spherocytosis
