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What is macrocytic and what type of anemia does it describe?
- Macrocytic = abnormally large RBCs
- B12/Folate deficiency anemia
What is the etiology of vitamin B12/folate anemia?
decreased intake or increased demand
Describe the pathophys of folate/B12 anemia.
Both folate and B12 are required for DNA synthesis in the RBC. Lack of either or both substances results in prolonged RBC maturation in bone marrow with over production of hemoglobin and abnormally large cells (megaloblasts). These large RBCs die prematurely in circulation.
Describe Pernicious Anemia.
Vitamin B12 deficiency anemia due to inadequate intrinsic factor (produced in stomach and needed for B12 absorption in ileum).
List the four P's of B12/Folate anemia.
- Peripheral neuropathy
- Pyramidal tract signs
- Posterior column degeneration
What is the psychosis associated with B12/Folate anemia also called?
What are potential complications of B12/Folate anemia?
Neurological disorder may be permanent if not controlled early.
Describe peripheral neuropathy associated with B12/Folate deficiency.
numbness or pain in the hands and feet
Describe the posterior column degeneration associated with B12/Folate deficiency.
Loss of fine touch or fine sensation
Describe the pyramidal tract signs associated with B12/Folate deficiency. What test is commonly used to detect pyramidal tract problems?
- weakness of muscles
- loss of muscle function
- test = Babinski's Reflex - stroke the bottom of the foot, if the toes curl upwards there is a problem. This reflex should be gone by age 2.
What is MS caused by?
Autoimmune disorder of the myelin sheaths.
What are normal Hgb levels?
Hemoglobin: 12 -18 g/dl
What are normal hct numbers?
What are the normal RBC values:
4.1 - 6.0 x 106
What are the normal mean corpostule volume (MCV) values?
What are the normal platelet values?
Platelets - 150-400 x 103
What are two common terms associated with iron anemia?
macrocytic and hypochromic
Describe the pathophys of iron deficiency.
Iron is necessary for cellular maturation and proliferation and hemoglobin synthesis. Inadequate availability results in decreased numbers, smaller size (microcytic), and less hemoglobin (hypochromic).
What are general signs of iron deficiency?
- Koilonychia: brittle, thin nails, shaped upwards like spoon
- red, burning tongue with loss of papillae
Where are papillae located?
tongue (taste buds)
Is sickle cell disease a single-gene or X-linked disorder? Is it dominant or recessive?
Recessive Single Gene disorder.
Where is the abnormal gene in sickle cell located? Instead of a glutamic amino acid in hemoglobin A, what is there?
Instead of glutamic acid there is a valine acid this makes it Hemoglobin S instead of hemoglobin A which it's supposed to be.
What triggers the problems of hemoglobin S? What triggers the sickling of the cell?
Hemoglobin S is fine unless there is not enough oxygen. Polymerization causes the sickling of the cell.
What is the degree of sickling dependent upon?
how little O2 there is, the lowering of pH, dehydration, and the amount of HbS
What does the sickling of the cell due to the flow of blood?
Sickling increases viscosity and slows blood flow which increases the time of exposure to the hypoxic environment. That increases sickling even more leading to vascular occlusion and infarction of tissue.
What is an infarct?
An area of necrosis in a tissue or organ resulting from obstruction of the local circulation by a thrombus or embolus.
What are common signs of sickle cell anemia?
- leg ulcers (skin manifestations)
- joint and skeletal problems, arthritis
- renal complications
- priapism (long, painful erection)
- GI issue
With sickle cell anemia, would you see an increased or decreased amount of reticulocytes (immature RBC)?
List the three common complications of sickle cell anemia.
- Vaso-occlusive crisis
- Aplastic crisis
- Hemolytic crisis
Describe Vaso-occlusive crisis
Hallmark of sickle cell anemia. Caused by occlusion of small blood vessels by sickled cells and results in ischemia and infarction to tissues downstream; precipitated by infection, fever, acidosis, dehydration, and exposure to cold
Describe Aplastic crisis.
Seen in Sickle cell anemia - usually associated with infection which causes temporary marrow aplasia; marrow overworked by stress of constant demand for new RBC; this is drastic
Describe hemolytic crisis.
Seen in sickle cell anemia. acute exacerbation of anemia; can lead to infarction and fibrosis of spleen (autosplenectomy).
What is autosplenectomy?
infarction and fibrosis of spleen
What is aplastic anemia?
- A = without
- plastic = plasia = growth/development
- Decrease in functional bone marrow resultinn in low RBC, platelet and WBC
- - can be familial or acquired. If acquired, probably through radiation or bone marrow trauma
What is hemophilia? What is the difference between Hemophilial A and B?
The most common inherited coagulation disorder. More severe if inherited. hemophilia A is due to deficit of factor 8 and Hemophilia B is due to factor 9.
What is thrombocytopenia?
Disorder of low platelets so the blood cannot clot properly.
List the 4 main etiologies of Thrombocytopenia.
- Decreased platelet production
- Decreased platelet survival
- Splenic Sequestration (pooling)
- Platelet Dilution
List causes of Decreased Platelet Production that can lead to thrombocytopenia.
- B12/Folate deficiency
- Radiation or chemotherapy
- Aplastic anemia
- Bone marrow cancer
List causes of Decreased Platelet Survival that can lead to thrombocytopenia?
- prosthetic heart valves
- circulating immune complexes
- increased destruction in the spleen
- disseminated intravascular coagulation
What are two causes of splenic sequestration (pooling) that can lead to thrombocytopenia.
What is a cause of platelet dilution that can lead to thrombocytopenia?
massive transfusions of blood that have been stored for more that 24 hours.
When do clinical manifestation of platelet deficiencies appear?
When the platelet count is under 100,000.
What clinical manifestations could appear when the platelet count drops to 50,000?
- increased bruising
- prolonged bleeding
What clinical manifestations could appear when the platelet count drops to 20,000?
spontaneous mucosal, deep tissue, and intracranial bleeding
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