APLASTIC ANAEMIA -
Noun: Aplastic Anaemia is a rare but extremely serious disorder that results when the bone marrow , the spongy interior of the bones, fails to producing red and white blood cells and platelets. As a result, the bone marrow contains large numbers of fat cells instead of the blood producing cells which would normally be present. Aplastic Anaemia is not a cancer, but because the disorder is treated like many cancers of the blood, most patients are seen at a cancer centre.

Here we see a sample of bone marrow in a patient with Aplastic Anaemia. Notice there are very few cells except for the fat cells.See Bone Marrow to compare this with the picture there.

The disease in most cases is acquired, that is, it is not inherited and is not present from birth, although there is a rare inherited form of the disease called Fanconi Anaemia .

Aplastic anaemia may be a consequence of use of high dose drugs and radiotherapy in treatment of cancer. There is usually prompt recovery when the drug or radiation treatment is stopped.

The disease may affect people of any age but there are peaks of incidence in young adults and in people over the age of 60 years.

In the remaining cases there is evidence of exposure to some factor which is known to cause damage to bone marrow stem cells. Aplastic anaemia may occur in pregnancy, but this is extremely rare. These cases usually resolve with the end of the pregnancy.

Certain diseases may, rarely, lead to aplastic anaemia. These include:

• viral hepatitis
• herpes and HIV
• disorders of the immune system
• blood disorders

In 50% of the people with aplastic anaemia, the cause is unknown. In other cases, the anaemia may be caused by

• aeroplane glue
• chemicals, such as benzene and pesticides
• chemotherapy
• drugs, such as chloramphenicol
• radiation therapy

Some experts believe that aplastic anaemia is an autoimmune disorder, a condition in which the body produces antibodies against its own blood cells. For this reason, drugs which kill off the white blood cells which are attacking the stem cells is often used to treat Aplastic Anaemia. It is called Immunosuppressive Therapy . Response rates using this treatment are currently 70% to 80%.

Any patient diagnosed with severe aplastic anaemia should have rapid HLA tissue typing performed to identify possible marrow donors, says Rainer Storb, MD. Blood transfusions from prospective marrow donors should be avoided and all blood transfusions should be irradiated.

In the mid 1970's there is a report that only 20% of patients with severe aplastic anaemia who were treated with transfusions and antibiotics alone survived. Half of the deaths occurred within 4 months of diagnosis! I have found out that their main problem was that they were not given platelets which is not a problem today.

I had conversations with Professor Ted Gordon-Smith and Dr. Judith Marsh who are both experts in the field of Aplastic Anaemia working and doing research at the Ruth Myles Unit at St George's Hospital in London. Their preferred treatment for adults, especially those in their 40's and up is the Immunosuppressive therapy even if the patient has a complete bone marrow match with a brother or sister. There is more of a risk of getting GVHD in older patients. I was told that chances of the getting Myelodysplasia or Leukaemia after the immunosuppressive treatment was about 5%. Chances of getting PNH was 15% - 20% but PNH was not life threatening or particularly debilitating. If the Immunosuppressive therapy did not work out (there is a 70% to 80% success rate with it) it could be done again or a couple times more. If there were allergic reactions to the ALG, which sometimes happens, or the treatment just was not working, one could then go on to have a Bone Marrow Transplant which the Professor stated had improved chances of success with those who had undergone the Immunosuppressive therapy. If the Bone Marrow Transplant is done first, the stem cells are so depleted in the course of the Cyclophosphamide preparative regimen that should there be a graft rejection or reason for it not to work, the Immunosuppressive therapy would not be as successful as there would be less stem cells to work with. According to the EBMT and IBMTR of which Dr. Marsh is a Council member, children have success rates of 90% with the Bone Marrow Transplants. This decreases as one gets older. In Europe, she tells me they will not do a BMT on anyone over 40. The downside of this is that with Immunosuppressive Therapy alone, one never does get back to 100% and there is almost always an underlying abnormality with the cells. This must be monitored to watch for any cellular progression to MDS or Leukaemic conditions so it is picked up early. But even if it does progress to these, I was told that a new technology called a mini-graft is very successfull in bone marrow transplants on these diseases using a much lower dose of chemotherapy.

So once the diagnosis is complete, (in order to increase one's options) there is an urgency to:

•Ensure you get HLA tissue typed as soon as possible. This is a rare disease and your Dr. may not be aware of the urgency, or if under the NHS (National Health Service), be so busy, this does not get the priority it should have.

•If you have them, get your brothers and sisters HLA tissue typed to see if any match.

•If no brothers or sisters, to start looking for a potential donor so you have that as an option should you decide to go for a bone marrow transplant or if the immunosuppressive therapy is not successful. Keep in mind that this is much more risky than a BMT from a brother or sister. Also keep your age in mind as stated above. Read GVHD . See Haplo transplant .

•Discuss with your Dr. the pros & cons of the Immunosuppressive therapy with relation to the bone marrow transplant .

•Do everything possible to move things along keeping in mind that the chances of success of transplantation can be lowered by a prolonged interval between diagnosis and transplantation, or if the patient has received multiple blood transfusions or has experienced serious infections.

•Keep a record of Dr. visits, sick leave forms, correspondence with doctors and Social Security. A diary is also useful. Make applications for Disability Benefit and Incapacity Benefit if applicable.

Preventive Measures

There are measures patients with low white blood cell counts can take to limit the risk of developing an infection. See Clean Diet and Program 2: Increase Hygiene

• Avoid exposure to crowds or to people with colds or contagious diseases.
• Practice good hygiene, including frequent hand washing.
• Make a practice of taking your temperature if you are having a poorer day than normal. The reason might be due to some internal infection. Your body will tell you by raising it's temperature above 37.5°C in an effort to handle it. Contact your doctor if it is above 37.5°C , certainly if it is above 38°C.
• Brush teeth regularly, bathe or shower daily; and pay special attention to hard-to-clean areas such as skin folds and the rectal area.
• Avoid cuts and scrapes. Patients with AA who think they might get injured while at work or engaging in hobbies or sports activities, should ask their health care provider about ways to protect themselves.
• If you notice some infection such a cut that is inflamed, gum or tooth infection, or your temperature goes high (37.5° C +) report this to your doctor ASAP so he can evaluate your situation and give you the correct antibiotics if needed.
• If your neutrophil count is less than .3 (some doctors have said .5) you should be in an isolation ward when you are in the hospital. Therefore it makes sense that when you leave the hospital and go home you should treat your home as an isolation ward as well. The soil and plants inside your house and outside are a source of the fungus aspergillus which grows in decaying vegetation. Get rid of plants and fresh flowers in your house and limit your time outside as the aspergillus spores are usually present in the air all year round.
• Showers should be run hot for at least 5 minutes each week if not normally used to eliminate bacterial growth in them and grouting around shower kept clean to prevent mould.
• Kitchen and bathroom sinks and bath tubs should be cleaned with soap and water or a disinfectant and dried thoroughly after each use.
• Use liquid soap in the bathroom. The bars of soap often remain wet and are a breeding place for dangerous bacteria.

Some infections can be prevented and almost all infections can be treated. People with aplastic anaemia should be a full partner with the health care team by taking care of themselves and reporting any problems they may have.

Low Platelet Count

Another type of blood cell affected by the AA patient are the platelets . Platelets help the blood to clot. If platelets are deficient, patients may develop bruises, may bleed easier than usual, or have trouble stopping the bleeding. For example, patients might notice blood on their toothbrush from bleeding gums or might develop nosebleeds. Women may experience heavy menstrual periods. Some people get tiny, red spots on their skin, especially on the arms or legs. These are called petechiae . Blood in the urine and stool sometimes occurs.

Symptoms and Treatment

Symptoms you should report to your doctor or hospital immediately include:

• Unusually heavy or prolonged bleeding anywhere
• Severe headache or visual changes
• Stiff neck

Doctors may order a platelet transfusion when the platelet count is low or to prevent or stop excessive bleeding. For this treatment, platelets usually are given through a needle inserted into a vein. Patients receive transfusions during an outpatient visit to the hospital, usually lasting several hours.

Preventive Measures

Safety measures to take include.

• Avoid activities that can result in bruising or bumping.
• Put off the use of sharp instruments such as tools, nail trimmers, razors or knives.
• Wear hard-sole shoes, gloves, or thick pants.
• Ask health care providers about the use of special sponge toothbrushes for people who have problems with gum bleeding. Use them if it is recommended.
• Some medications can affect clotting ability. It is important to avoid medications that contain aspirin or aspirin-like products (for example, Motrin, ibuprofen, or other anti-inflammatory drugs) unless the doctor has instructed otherwise as these reduce platelet count. Ibruprofen and others may also cause bleeding in the stomach which can be serious if your platelet levels are very low.
• Remember to report any signs that platelet counts might be low, such as easy bruising, nose or gum bleeding, blood in urine or petechiae (tiny red spots on arms or legs).

Low Red Blood Cell Count

The red blood cells carry oxygen to all parts of the body. When red blood cells are not being produced as is the case in Aplastic Anaemia, transfusions will be required until the condition is handled.

Symptoms and Treatment

Without enough oxygen, patients may feel short of breath or feel their heart is pounding very fast. As a result, people with AA may feel tired and look pale. If these side effects are severe, inform your doctor right away. Transfusions of red blood cells may be prescribed to help these symptoms. Red cell transfusions are matched by blood types. First, a sample of the patient's blood is sent to the blood bank to identify its types: A, B, O, AB. Each type is either Rh positive or negative. This is called "typing the blood." A small sample of the donor blood is then matched to the blood type. A transfusion of only red blood cells, called a "unit" of packed RBCs (red blood cells), is given to the patient through a needle in the arm. Transfused blood should be leucocyte depleted and irradiated.

Preventative Measures

• It may be beneficial to sleep more and rest between activities so the body can conserve energy.
• Light exercise such as walking may give you more energy.
• Spend energy on accomplishing important tasks.

AA patients who need dental work should discuss this with their doctor. Infection and/or bleeding could be problems if dental work is done when blood counts are low. You may need to be topped up with red blood and platelets if a tooth has to be pulled. Your doctor may want to see you after the dental work for a blood test in case of a dental infection getting into the blood system.

It has been stated in many medical reports on AA & MDS that there is a possibility of the graft being rejected if a large number of transfusions are received prior to the BMT. The transfused blood and platelets may be recognised as antigens by the new white cells being produced by the donors stem cells resulting in GVHD. According to Professor Gordon-Smith and Dr. Judith Marsh this risk has been drastically reduced recently with modern technology. The problem was that white blood cells were in the transfused blood causing reactions. Transfused blood today has been effectively filtered to remove all white blood cells which might cause this problem. However, many red blood transfusions can cause another problem called Haemosiderosis .

Aplastic anaemia patients who do not receive a bone marrow transplant continue to have an underlying abnormality in the bone marrow and may go on to develop myelodysplasia or a disease called Paroxysmal nocturnal haemogloburinaemia . Both of these can progress to leukaemia .

Greek - a = not + plássein = to form

Greek - an = without + heîma = blood

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