Anemia

Anemia: Anemias are marked by abnormally low numbers of RBC (red blood cell), a deficiency of hemoglobin, or a low volume of packed RBCs per 100 ml of blood, stemming form an imbalance between blood production and loss through injury or bleeding.  Such disorders include aplastic anemia, folic acid deficiency anemia, sickle cell anemia, iron deficiency anemia, and pernicious anemia.

Aplastic anemia also called hypoplastic anemia:  results from a deficiency of all of the blood's formed elements, caused by the bone marrow's failure to generate an adequate supply of new cells.  Aplastic anemia usually develops when damaged or destroyed stem cells inhibit RBC production.  It also develops when damaged bone marrow microvasculature impairs cell growth and maturation.

Often used interchangeably with other terms for bone marrow failure, aplastic anemia properly refers to pancytopenia resulting from the decreased functional capacity of a hypoplastic, fatty bone marrow.  Two forms of idiopathic aplastic anemia are :  congenital hypoplastic anemia (anemia of Blackfan and Diamond), which develops between ages 2 months and 3 months; and Fanconi's syndrome, in which chromosomal abnormalities are usually associated with multiple congenital anomalies ; such as dwarfism and hypoplasia of the kidneys and spleen. Mortality for aplastic anemia with severe pancytopenia is 80% to 90%.  Death may result from bleeding or infection.

Cause:  Aplastic anemia may result from drug use, toxic agents, such as benzene and chloramphenicol., radiation, suspected but unconfirmed immunologic factors, severe disease such as hepatitis, preleukemia and neoplastic infiltration of bone marrow, congenital abnormalities, or induced change in the development of the fetus (suspected as a cause in the absence of a consistent familial or genetic history of aplastic anemia).

Symptoms:

Symptoms vary with the severity of pancytopenia, often develop insidiously, and may include the following signs and symptoms:

Progressive weakness
Fatigue
Shortness of breath
Headache
Pallor
Tachycardia and congestive heart failure
Eccymosies
Petechiae
Hemorrhage, especially from the mucous membranes (nose, gums, rectum, vagina) or into the retina or central nervous system

Treatment:

Eliminate any identifiable cause and provide vigorous supportive measures, such as packed RBC, platelet, and experimental HLA matched leukocyte transfusions.  Even after elimination of the cause, recovery can take months.  Bone marrow transplantation is the preferred treatment for anemia stemming from severe aplasia and for patients needing constant RBC transfusions.

Patients with low WBC counts may need reverse isolation to avoid infection.  The infection itself may require specific antibiotics; however, these are not given prophylactically because they tend to encourage resistant strains of organisms.  Patients with low hemoglobin counts may need respiratory support with oxygen, as well as blood transfusions.

Corticosteroids - to stimulate erythroid production (successful in children, unsuccessful in adults)

Marrow stimulating agents, such as androgens

Immunosuppressive agents (if the patient does not respond to other therapy)

Note:  report any signs of infection to our doctor promptly.

Pernicious anemia

Pernicious Anemia also called Addison's anemia: progressive, megaloblastic, macrocytic anemia primarily affecting persons of northern European ancestry.  Onset is typically between ages 50 and 60, incidence rises with increasing age.  Pernicious anemia causes serious neurologic, gastric, and intestinal abnormalities.  Untreated, in may lead to permanent neurologic disability and death.

Cause: results from a deficiency of vitamin B12, which may result from a genetic predisposition or an inherited autoimmune response.

Symptoms:

Pernicious anemia has an insidious onset but eventually causes an unmistakable triad of symptoms including:

Weakness
Sore tongue
Numbness and tingling in the extremities
Pale lips, gums, and tongue and faintly jaundiced sclerae also occur.

Systemic signs may include: Pale to bright yellow skin and indications of infection especially of the genitourinary tract.

GI symptoms:  nausea, vomiting, anorexia, weight loss, flatulence, diarrhea, and constipation.  Gingival bleeding and tongue inflammation may hinder eating and intensify anorexia.

CNS symptoms:  neuritis; weakness in extremities; peripheral numbness and paresthesias; disturbed position sense; lack of coordination; ataxia; impaired fine finger movement; positive Babinski's and Romberg's signs; lightheadedness; altered vision; optic muscle atrophy; loss of bowel and bladder control; irritability, poor memory, , headache, depression, and delirium; and, in males, impotence.  Some of these symptoms are temporary, irreversible CNS changes may have occurred before treatment.

Cardiovascular symptoms:  weakness, fatigue, light headedness, palpitations, wide pulse pressure, dyspnea, orthopnea, tachycardia, premature beats, and, eventually, congestive heart failure.

Treatment:

B12 injection

Iron replacement

May require blood transfusions, digitalis, a diuretic, and a low sodium diet for CHF.

May require antibiotic therapy

Iron Deficiency Anemia:

Iron deficiency anemia:   Caused by an inadequate supply of iron for optimal information of RBCs, this anemia results in smaller cells with less color on staining.  Body stores or iron, including plasma iron, decline, as do levels of transferring, which binds with and transports iron.  Insufficient body stores of iron lead to a depleted RBC mass an, in turn, to a diminished hemoglobin concentration and oxygen-carrying capacity of the blood.

Iron deficiency anemia occurs most commonly in premenopausal women, infants (particularly premature or low birth weight infants), children, and adolescents (commonly girls).

Cause:  inadequate dietary intake of iron (less that 1 mg/day).  This may occur during prolonged unsupplemented breast or bottle feeding of infants or during periods of stress, such as rapid growth in children and adolescents.  It may also result from iron malabsorption caused by chronic diarrhea, partial or total gastrectomy, and malabsorption syndromes such as celiac disease.

Symptoms:

Fatigue
Listlessness
Pallor
Inability to concentrate
Dyspnea on exertion
Irritability
Headache

For chronic iron deficiency:  may develop brittle, spoon shaped nails and cracks at the corners of the mouth.

Treatment:

To determine the underlying cause of anemia

Oral iron and ascorbic acid

In some cases iron may be administer parenterally

Increase iron in diet

Folic Acid Deficiency Anemia

Folic Acid Deficiency Anemia:  A slowly progressive, megaloblastic anemia, this common disorder occurs most often in infants, adolescents, pregnant and lactating females, alcoholics, older adults, and in persons with malignant or intestinal diseases.

Cause:  May result from alcohol abuse (alcohol may suppress metabolic effects of folate); poor diet; impaired absorption; or bacteria competing for available folic acid.

Other causes can include excessive cooking, which can destroy  a high percentage of folic acid in foods; limited storage capacity in infants; prolonged drug therapy; and increased folic acid requirement during pregnancy, during rapid growth in infancy

Symptoms:

Progressive fatigue
Shortness of breath
Palpitations
Weakness
Glossitis
Nausea
Anorexia
Headache
Fainting
irritability
Forgetfulness
Pallor
Slight jaundice

Treatment:

Folic acid supplement and elimination of contributing causes.

Definition- anemia is a reduced number of circulating red blood cells or a decrease in quality or quantity of hemoglobin (the part of a red blood cell that carries oxygen, it is primarily made up of iron).

Classification:
The most common classification system is based on the cell structure.  This system focuses on the cells size and the content of hemoglobin.  When describing cells "cytic" refers to the cell size, and  "chromic" is used to describe hemoglobin content. In some animas the cell has different shapes, this is referred to as poikilocytosis. There are 3 major types of anima, caused by the above mentioned cell abnormalities.

Macrocytic-normochromic:
Macro means large and normo means normal, so this is a large cell, with normal contents. This problem is usually caused by defective DNA ( deoxyribonucleic acid), which is like 1/2 of the brain of the cell, telling it what to do.Not only are the cells unusually large in diameter, they also have increased thickness and contents.  This defect is usually caused by a lack of vitamin B12 or folate.  These are required by the cell for proper growth and multiplication.  While the DNA is maturing at a slower rate than normal the other 1/2 of the brain is working normally, but has longer to work.  This causes  more contents and contributes to the cell becoming larger and the increased thickness.  Due to their increased size and their inability to change shape readily ( to go through capillary beds, the most narrow part of the system of pipes used for blood to travel through the body) they have a shorter than normal life.  This causes a decrease in the number of red blood cells in the system.  The body is unable to make enough new red blood cells (RBC), to replace those being lost.  There are 2 types of anemia with this type of problem.

Pernicious anemia- which is caused by a lack of vitamin B12.

Treatment- usually B12 injections  to correct the deficiency.

Folate deficiency anemia- which is caused by a lack of folate.

Treatment-can be treated with diet if not severe, medication if more severe.

Microcytic-hypochromic anemia:
Micro means small and hypo means less than normal, so we have a small cell with less contents.  Their smaller size allows them to  go through the system of pipes at a higher speed, which damages them and they have shorter lives.  They also have less hemoglobin, which decreases the amount of oxygen they can carry.  This condition can be caused by a variety of conditions. Iron must be broken down into smaller pieces to be used by the body.  If the iron is not being broken down, the RBC has none available for use.

Iron deficiency anemia- lack of iron for hemoglobin production, this is usually caused by blood loss over a long period of time. The iron is used up in an attempt to make new RBC's. Another cause is pregnancy, with an increased need for RBC's for the fetus.

Treatment- Stop the bleeding and give more iron through diet or medication.

Sideroblastic anemia (SA) - cell unable to use iron available, it is not being broken down.  This is caused by the iron not being used by the body to make hemoglobin.  There are 2 reasons this may happen.

First, it can be inherited from a parent.

Second, it can be acquired.  The main cause of this is unknown.

Treatment- Pyridoxine therapy has been effective for both types.  If Pyridoxine is effective, lifelong use of it is required.  If it is not effective there is a chance of death.

Thalasemia - There are two causes. Impaired production of hemoglobin is one. In the other, RBC's are being attacked by the body's defense system because it does not recognize them.  Both are caused by a congenital genetic defect.

Normocytic-normochromic:
The cells and their contents are normal, but there are not enough of them.  These animas  are less common than the other 2 types.  There are 5 types that share only the normal size and normal contents.  They are caused by different problems and have different treatments as well as outcomes.

Aplastic Anemia - In the first stage, RBC's fail to develop completely, not at all, or they are defective.  This can be caused by congenital abnormalities or by a large number of chemicals, drugs, and radiation.

Hemolytic Anemia - Mature RBC's are destroyed prematurely.

Sickle Cell Anemia - The RBC's are abnormally shaped and the hemoglobin is also abnormal.  This causes the RBC's to die prematurely.  The abnormal shape has been selectively saved in a few parts of the world.  People with Sickle Cell are not affected by malaria. This has to do with the shape of the RBC's, they look like saucers rather than round. This one redeeming quality is not a good trade off.  People often die from this disease depending on how many of their cells are abnormal. Large numbers of RBC's dying at the same time can damage the liver and the kidneys.

Treatment - Blood transfusions can be effective. Click here for more on Sickle Cell Anemia.

Posthemmorrhagic Anemia - This is caused by blood loss over a period of time.  This causes an increased need for new RBC's, which will cause the body to run out of iron, because there is a limited amount of iron and is frequently the factor that limits new RBC production.  The body can not function without iron, either in reserve or in the blood system.

Treatment- Blood transfusions can be effective.

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