Aplastic anemia

Aplastic anemia

Medical description

Marie Curie and Eleanor Roosevelt, among others, suffered from this very serious and rare disease. Aplastic – or aplastic – anemia occurs when the bone marrow no longer produces enough hematopoietic stem cells. However, these are the source of all blood cells, of which the three types are: red blood cells, white blood cells and platelets.

Aplastic anemia therefore causes three categories of symptoms. First, those which are common to the different types of anemia: either the signs of a deficiency in red blood cells – and therefore of a deficient transport of oxygen. Then, the symptoms associated with the lack of white blood cells (vulnerability to infections), and finally, the lack of blood platelets (coagulation disorders).

It is a very rare form of anemia. Depending on the case, it is acquired or inherited genetically. This disease can appear suddenly and last a short time or become chronic. Once almost always fatal, aplastic anemia is now much better treated. However, if it is not treated quickly, it will get worse and lead to death. Successfully treated patients are more likely to develop other illnesses later, including cancer.

This disease can occur at any age and affects both men and women (but it is usually more serious in men). It seems to be more common in Asia than in the United States or Europe.

Causes

In 70% to 80% of cases6, the disease has no known cause. It is then said that it is a primary or idiopathic aplastic anemia. Otherwise, here are the factors that may be responsible for its occurrence:

– Hepatitis (5%)

– Medicines (6%)

  • Sels d’or
  • Sulfamidés
  • chloramphenicol
  • Non-steroidal anti-inflammatory drugs
  • Anti thyroid drugs (used in hyperthyroidism)
  • Phenothiazines
  • Penicillamine
  • Allopurinol

– Toxins (3%)

  • Benzene
  • Canthaxanthine

– Fifth disease – “foot-hand-mouth” (parvovirus B15)

– Pregnancy (1%)

– Other rare cases

It is important to differentiate plastic anemia from other diseases that are similar to it. Indeed, this syndrome is different from the anemias found in certain cancers and their treatment.

There is an inherited form of aplastic anemia called “Fanconi anemia”. In addition to suffering from aplastic anemia, people with this extremely rare condition are shorter than average and have various birth defects. Usually, they are diagnosed before the age of 12 and many do not reach adulthood.

Symptoms of the disease

  • Those linked to a low level of red blood cells: pale complexion, fatigue, weakness, dizziness, rapid heartbeat.
  • Those linked to a low level of white blood cells: increased susceptibility to infections.
  • Those related to a low level of blood platelets: easily bruised skin, abnormal bleeding from the gums, nose, vagina or gastrointestinal system.

People at risk

  • This disease can appear at any age, but it is most often seen in children, adults around the age of 30 and people over 60.
  • There may be a genetic predisposition as in the case of Fanconi anemia.

Risk factors

Aplastic anemia is a rare disease. People who are exposed to different causes of the disease (see Causes above) increase their risk of developing it, to varying degrees.

– Prolonged exposure to certain toxic products or to radiation.

– Use of certain drugs.

– Certain physical conditions: diseases (leukemia, lupus), infections (hepatitis A, B, and C, infectious mononucleosis, dengue), pregnancy (very rarely).

Prevention

Avoiding prolonged exposure to toxics or drugs mentioned above is a valid precaution at all times – and not just to prevent aplastic anemia. However, in most cases, the initial onset of the latter cannot be prevented. On the other hand, when we know the origin of an anemia, it is possible to prevent its recurrence by avoiding exposure to one or other of the following factors if they are involved:

– toxic products;

– high-risk drugs;

— radiations.

In the event of aplastic anemia due to hepatitis, it is a question of applying the recommended measures to prevent the different types of hepatitis. See the Hepatitis sheet.

In severe aplastic anemia, the doctor sometimes prescribes antibiotics to prevent bacterial infections.

Medical treatments

The disease is rare and has a high potential for complications. The care will be given by a doctor specializing in the field, most of the time with a multidisciplinary team and in an ultra-specialized center.

  • In the first place, it will be necessary to stop the taking of drugs possibly responsible for the anemia.
  • Antibiotics will be needed for the prevention and treatment of any infection.
  • The combination of anti-thymocyte globulins for 5 days, cortisone and cyclosporin can, in some cases, induce remission of the disease7.

The combination of anti-thymocyte globulins for 5 days, cortisone and cyclosporine can in some cases induce remission of the disease

Special care. For people with aplastic anemia, certain precautions are necessary in everyday life:

– Protect yourself from infections. It is important to wash your hands often with antiseptic soap and to avoid contact with sick people.

– Shave with an electric razor rather than a blade to avoid cuts. Since aplastic anemia is associated with a low level of blood platelets, the blood clots less well and blood loss should be avoided as much as possible.

– Prefer toothbrushes with soft bristles.

– Refrain from practicing contact sports. For the same reasons as those mentioned above, it is necessary to avoid any occasion of blood loss, and therefore of injury.

– Also avoid doing too intensive exercises. On the one hand, even light exercise can cause fatigue. On the other hand, in the event of prolonged anemia, it is important to spare the heart. This has to work a lot more because of the deficient oxygen transport associated with anemia.

The doctor’s opinion

As part of its quality approach, Passeportsanté.net invites you to discover the opinion of a health professional. Dr Dominic Larose, emergency physician, gives you his opinion on aplastic anemia :

This is an extremely rare situation for which you will need to consult a specialist doctor for appropriate treatment. Most general practitioners will see only one case in their career, if at all.

Dr Dominic Larose, M.D.

 

Complementary approaches

There is no natural treatment that has specifically been the subject of serious studies in the case of aplastic anemia.

According to the Aplastic Anemia & MDS International Foundation, the use of herbal remedies and vitamins could worsen the disease and obstruct processing. However, she recommends a Healthy eating to optimize blood production.1

It is also advisable to join a support group.

Landmarks

Canada

Aplastic Anemia and Myelodysplasia Association of Canada

This site provides support and information to patients and families. In English only.

www.amamac.ca

 

United States

Aplastic Anemia & MDS International Foundation

This American site with an international vocation is multilingual and it should soon include a section in French.

www.aplastic.org

Fanconi Anemia Research Fund, Inc

This English site is intended for people with Fanconi anemia and their families. In particular, it provides access to a PDF manual entitled “Fanconi Anemia: a Handbook for Families and Their Physicians”.

www.fanconi.org

 

 

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