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Wednesday, April 22, 2009

Blood Disorders

Blood Disorders

There are many types of blood disorders. These include:

  • Anemia
  • Haemophilia
  • bleeding disorders


Anemia

Anemia is defined as a qualitative or quantitative deficiency of hemoglobin, a molecule found inside red blood cells (RBCs). Since hemoglobin normally carries oxygen from the lungs to the tissues, anemia leads to hypoxia (lack of oxygen) in organs. Since all human cells depend on oxygen for survival, varying degrees of anemia can have a wide range of clinical consequences. Anemia is caused by the lack of iron in the body as well.

The three main classes of anemia include excessive blood loss (acutely such as a hemorrhage or chronically through low-volume loss), excessive blood cell destruction (hemolysis) or deficient red blood cell production (ineffective hematopoiesis).

Anemia is the most common disorder of the blood. There are several kinds of anemia, produced by a variety of underlying causes.

There are two major approaches of classifying anemias, the "kinetic" approach which involves evaluating production, destruction and loss, and the "morphologic" approach which groups anemia by red blood cell size. The morphologic approach uses a quickly available and cheap lab test as its starting point. On the other hand, focusing early on the question of production may allow the clinician more rapidly to expo se cases where multiple causes of anemia coexist.


Signs and symptoms

Anemia goes undetected in many people, and symptoms can be small and vague. Most commonly, people with anemia report a feeling of weakness or fatigue in general or during exercise, general malaise and sometimes poor concentration. People with more severe anemia often report dyspnea (shortness of breath) on exertion. Very severe anemia prompts the body to compensate by increasing cardiac output, leading to palpitations and sweatiness, and to heart failure.

Pallor (pale skin, mucosal linings and nail beds) is often a useful diagnostic sign in moderate or severe anemia, but it is not always apparent. Other useful signs are cheilosis and koilonychia.Pica, the consumption of non-food such as dirt, paper, wax, grass, ice, and hair, may be a symptom of iron deficiency, although it occurs often in those who have normal levels of hemoglobin.

Chronic anemia may result in behavioral disturbances in children as a direct result of impaired neurological development in infants, and reduced scholastic performance in children of school age.



Treatments for anemia

There are many different treatments for anemia and the treatment depends on severity and the cause.

Iron deficiency from nutritional causes is rare in non-menstruating adults (men and post-menopausal women). The diagnosis of iron deficiency mandates a search for potential sources of loss such as gastrointestinal bleeding from ulcers or colon cancer. Mild to moderate iron deficiency anemia is treated by iron supplementation with ferrous sulfate or ferrous gluconate. Vitamin C may aid in the body's ability to absorb iron.

Vitamin supplements given orally (folic acid) or subcutaneously (vitamin B-12) will replace specific deficiencies.

In anemia of chronic disease, anemia associated with chemotherapy, or anemia associated with renal disease, some clinicians prescribe recombinant erythropoietin, epoetin alfa, to stimulate red cell production.

In severe cases of anemia, or with ongoing blood loss, a blood transfusion may be necessary.

Blood transfusions for anemia

Doctors attempt to avoid blood transfusion in general, since multiple lines of evidence point to increased adverse patient clinical outcomes with more intensive transfusion strategies. The physiological principle that reduction of oxygen delivery associated with anemia leads to adverse clinical outcomes is balanced by the finding that transfusion does not necessarily mitigate these adverse clinical outcomes.

In severe, acute bleeding, transfusions of donated blood are often lifesaving. Improvements in battlefield casualty survival is attributable, at least in part, to the recent improvements in blood banking and transfusion techniques.

Transfusion of the stable but anemic hospitalized patient has been the subject of numerous clinical trials, and transfusion is emerging as a deleterious intervention.

Four randomized controlled clinical trials have been conducted to evaluate aggressive versus conservative transfusion strategies in critically ill patients. All four of these studies failed to find a benefit with more aggressive transfusion strategies.

In addition, at least two retrospective studies have shown increases in adverse clinical outcomes with more aggressive transfusion strategies.

Hyperbaric Oxygenation

Treatment of exceptional blood loss (anemia) is recognized as an indication for hyperbaric oxygen (HBO) by the Undersea and Hyperbaric Medical Society. The use of HBO is indicated when oxygen delivery to tissue is not sufficient in patients who cannot be transfused for medical reasons. HBO may be used for medical reasons when threat of blood product incompatibility or concern for transmissible disease are factors.


Haemophilia

Haemophilia is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation. In its most common form, Hemophilia A, clotting factor VIII is absent. In Haemophilia B, factor IX is deficient. Hemophilia A occurs in about 1 in 5,000–10,000 male births, while Hemophilia B occurs at about 1 in about 20,000–34,000.

The effects of this sex-linked, X chromosome disorder are manifested almost entirely in males, although the gene for the disorder is inherited from the mother. Females have two X chromosomes while males have only one, lacking a 'back up' copy for the defective gene. Females are therefore almost exclusively carriers of the disorder, and may have inherited it from either their mother or father. In about 30% of cases of Hemophilia B, however, there is no family history of the disorder and the condition is the result of a spontaneous gene mutation.

These genetic deficiencies may lower blood plasma clotting factor levels of coagulation factors needed for a normal clotting process. When a blood vessel is injured, a temporary scab does form, but the missing coagulation factors prevent fibrin formation which is necessary to maintain the blood clot. Thus a haemophiliac does not bleed more intensely than a normal person, but for a much longer amount of time. In severe haemophiliacs even a minor injury could result in blood loss lasting days, weeks, or not ever healing completely. The critical risk here is with normally small injuries which, due to missing factor VIII, take long times to heal. In areas such as the brain or inside joints this can be fatal or permanently debilitating.


Causes

Hemophilia is nearly always caused by a genetic error causing the lack of a normally functioning clotting factor:

  • Hemophilia A involves a lack of functional clotting Factor VIII.
  • Hemophilia B involves a lack of functional clotting Factor IX.
  • Hemophilia C involves a lack of functional clotting Factor XI.
  • Hypofibrinogenemia involves a lack of functional clotting Factor I.


Treatment

Though there is no cure for hemophilia, it can be controlled with regular infusions of the deficient clotting factor, i.e. factor VIII in haemophilia A or factor IX in hemophilia B. Factor replacement can be either isolated from human blood serum, recombinant, or a combination of the two. Some hemophiliacs develop antibodies (inhibitors) against the replacement factors given to them, so the amount of the factor has to be increased or non-human replacement products must be given, such as porcine factor VIII.

If a patient becomes refractory to replacement coagulation factor as a result of circulating inhibitors, this may be partially overcome with recombinant human factor VII (NovoSeven), which is registered for this indication in many countries.

It is recommended that people affected with Hemophilia do specific exercises to strengthen the joints, particularly the elbows, knees, and ankles. Exercises include elements which increase flexibility, tone, and strength of muscles, increasing their ability to protect joints from damaging bleeds. These exercises are recommended after an internal bleed occurs and on a daily basis to strengthen the muscles and joints to prevent new bleeding problems. Many recommended exercises include standard sports warm-up and training exercises such as stretching of the calves, ankle circles, elbow flexions, and Quadriceps sets.

Alternative and complementary treatments

Scientific studies indicate that hypnosis and self-hypnosis can be effective at reducing bleeds and the severity of bleeds and thus the frequency of factor treatment. Herbs which strengthen blood vessels and act as astringents may also benefit patients with hemophilia, however there is little or no peer reviewed research to support these claims. Recommended herbs include: Bilberry (Vaccinium myrtillus), Grape seed extract (Vitis vinifera), Scotch broom (Cytisus scoparius), Stinging nettle (Urtica dioica), Witch hazel (Hamamelis virginiana), and yarrow (Achillea millefolium).


Bleeding disorders

Bleeding disorders is a general term for a wide range of medical problems that lead to poor blood clotting and continuous bleeding. Doctors also call them terms such as coagulopathy, abnormal bleeding and clotting disorders.

Normally, if bleeding occurs, our body forms a blood clot which helps to stop bleeding. Our body need a special cell in order to form a clot. These special cells are called platelets and also proteins are included in blood clotting factors. Bleeding disorders occur when your body do not have had enough platelets or proteins for forming clot or they are not working properly.

Blood clotting, is the process that controls bleeding. It changes blood from a liquid to a solid. It's a complex process involving as many as 20 different plasma proteins, or blood clotting factors. Normally, a complex chemical process occurs using these clotting factors to form a substance called fibrin that stops bleeding. When certain clotting factors are deficient or missing, the process doesn't occur normally.

This causes them to bleed long-lasting than those whose blood factor levels are normal. It is a fact that persons with bleeding disorders can lead death from minor injuries or their blood flows faster. Bleeding problems can range from mild to severe.


Symptoms

Symptoms of bleeding disorders include:

  • Excessive bleeding
  • Easy bleeding
  • Nose bleeding
  • Abnormal menstrual bleeding (women)
  • Excessive bruising
Bleeding disorder risks include:
  • Scarring of the joints or joint disease
  • Vision loss from bleeding into the eye
  • Chronic anemia from blood loss. Anemia is a low red blood cell count
  • Neurologic or psychiatric problems
  • Death, which may occur with large amounts of blood loss or bleeding in critical areas, such as the brain.


Causes


Some blood disorders are running in a family and it affects as the generation develops. Other factors that can cause bleeding disorders are some illnesses which includes severe liver disease, vitamin k deficiency and also long-time use of antibiotics and use of anticoagulant drugs. Hemophilia and other very rare blood disorders are also included.

Some common causes of bleeding disorders:

  • von Willebrand's disease , which is an inherited blood disorder thought to affect between 1% and 2% of the population.
  • Immune system-related diseases, such as allergic reactions to medications, or reactions to an infection.
  • Cancer, such as leukemia, which is a blood cancer.
  • Liver disease.
  • Bone marrow problems.
  • Disseminated intravascular coagulation, which is a condition often associated with child bearing, cancer, or infection, in which the body's clotting system functions abnormally.
  • Pregnancy-associated eclampsia, also known as severe toxicity of pregnancy.
  • Antibodies, a type of immune system protein, that destroy blood clotting factors.
  • Medicines, such as aspirin, heparin, warfarin, and drugs used to break up blood clots.



Treatment

A method for treating patients suffering from bleeding disorders not caused by clotting factor defects or clotting factor inhibitors, as well as a novel composition for use in treating bleeding disorders as disclosed. The method includes administering to a patient a composition comprising an effective haemostatic amount of factor VIIa, and is particularly effective in treating patients suffering from thrombocytopenia and von Willebrand's disease, as well as other platelet disorders. A composition suitable for use in treating such bleeding disorders comprises purified factor VIIa in a concentration of at least 25 μg/ ml.



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