Anaemia

Anaemia Questions : •

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What is unusual about some of these red cells? What are the unusual cells called?

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cells are fragment ed

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burr cells, helmet cells, triangle cells

Bone marrow aspirate Questions : •

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How is an aspirate obtained? What are the cells with nuclei?

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Aspirates are obtained through a needle positione d usually into the posterior iliac crest (pelvis) or sternum (obviousl y more risky).

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The cells with nuclei are progenito r cells of the blood which divide and

differenti ate in the bone marrow. Note the nucleate red blood cell.

Anaemia due to red cell loss What is anaemia? Red blood cells contain haemoglobin which is an oxygen-carrying molecule. The red cells travel through the lungs where the haemoglobin becomes loaded with oxygen and bright red in colour. The red cells are then pumped by the heart to the rest of the body where the haemoglobin gives up its oxygen to the tissues and becomes dark blue in the process. 'Anaemia' means that the amount of red cells, and therefore of haemoglobin, in the blood is abnormally low. This causes the oxygen-carrying capacity of blood to be reduced.

What causes anaemia and what are the risk factors? Anaemia may be caused by excessive loss of blood, by destruction of red cells or by decreased red cell production. Only the first two of these causes will be discussed here (see separate factsheet for anaemia caused by decreased red blood cell production). Excessive loss of blood may be sudden:

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Accidents

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Childbirth

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Surgery

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A ruptured blood vessel

Excessive blood loss may also occur over a long period of time (chronic bleeding):

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Heavy menstruation

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Bleeding cancer or polyps (benign growths) in the gut

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Bleeding gastric or duodenal ulcers

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Nosebleeds

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Bleeding haemorrhoids (piles)

Increased destruction of red blood cells occurs in hereditary conditions in which either the haemoglobin molecules or the red cells themselves are abnormal:

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Thalassaemia (abnormal haemoglobin)

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Sickle cell disease (abnormal haemoglobin)

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Hereditary spherocytosis (abnormally shaped red cells)

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Hereditary elliptocytosis (abnormally shaped red cells)

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G6PD deficiency (lack of an enzyme in red cells)

These hereditary conditions are fairly common throughout the world but different types are more common in different races. Increased destruction of red blood cells also occurs in conditions in which the immune system produces antibodies (molecules normally produced to kill bacteria and viruses) that bind to and destroy red cells. This is called 'autoimmune haemolytic anaemia' and many people with this type of anaemia have an underlying condition such as an infection, primary autoimmune disease or leukaemia. Any condition in which the spleen is enlarged also causes destruction of red cells.

What are the symptoms and complications of anaemia? The symptoms will depend on the severity of the anaemia. Pallor (a pale complexion) is a poor indication of the degree of anaemia. If the excessive bleeding is chronic, such as from a stomach ulcer or heavy periods, there may be no symptoms at all or there may be:

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Tiredness

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Faintness

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Dizziness (especially when standing)

If the blood loss is more severe or more rapid (as in an accident) there may be:

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Thirst

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Sweating

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Severe fatigue

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Breathlessness

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Chest pain

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Heart attack

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Stroke (due to lack of oxygen to the brain)

In people who have increased red cell destruction, the breakdown products of haemoglobin may cause jaundice (a yellow colouring of the skin and eyes).

How do doctors recognise anaemia? Anaemia is identified and defined by simple blood tests measuring the number of red blood cells and the amount of haemoglobin in the blood. Once somebody is found to be anaemic, there is a wide range of other tests which may be needed to find out the cause. In the case of sudden bleeding, the diagnosis is often obvious from the circumstances. If the bleeding is chronic, then investigations will be performed to find the site of the bleeding. This may include a careful menstrual history and examination of the stools and urine for blood. Subsequent examination of the intestines (for ulcers, polyps or cancers) or of the kidneys and bladder (for polyps or cancers) may be necessary. In the hereditary disorders, the diagnosis is often clear from the history of the complaint and the family background. Often 'electrophoresis' is performed. This is a blood test that detects the presence of abnormal haemoglobin. Sometimes a more detailed genetic analysis is necessary. Autoimmune haemolytic anaemia is diagnosed by the presence of antibodies in the blood that bind to red cells. Further investigation for an underlying condition will also be undertaken.

What is the treatment for anaemia? Self-care action plan

The most important thing is to avoid any situations that may provoke anaemia. If the anaemia is due to bleeding, this is often a difficult thing to predict but if you have any of the symptoms, you should see your doctor. If you have any of the hereditary anaemias or autoimmune haemolytic anaemia, avoid the triggers. These vary depending on the condition and should be discussed with your doctor. Medicines The treatment of anaemia due to excessive bleeding depends on the severity of the blood loss and the degree of anaemia. The best treatment for rapid blood loss or severe anaemia is blood transfusion, an oxygen mask and treating the source of the bleeding. Iron supplements are also taken because iron, which is needed to make haemoglobin, is lost during bleeding. If the bleeding is slow and the anaemia mild, simply treating the cause of the bleeding (with or without the addition of iron supplements) may be enough. Autoimmune haemolytic anaemia can sometimes be successfully treated with steroids.

The treatment of the hereditary anaemias, for which there is no readily available cure, is more complicated and will depend on the severity of the disease and the degree of anaemia. If there is a precipitating factor, such as infection, this will be treated.

Surgery If the underlying cause of excessive bleeding is amenable to a surgical procedure, such as repair of a ruptured blood vessel, this may be performed. In some of the hereditary anaemias, the spleen (an organ situated in the upper left part of the abdomen) may be removed if it becomes excessively large. This is because the spleen 'collects' and breaks down red blood cells: a larger spleen can take up more red cells, making the anaemia worse.

What is the outcome of having anaemia? The outcome of anaemia depends entirely on its cause. However, uncomplicated anaemia due to excessive bleeding, from a duodenal ulcer for example, is usually treated successfully. The hereditary disorders will be managed by a team of specialists who will be able to advise you on appropriate therapies and outcome at each stage of the disease.

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Thin blood smear, sickle cell anaemia. The red blood cells are distorted. Photo copyright ITM

AA/MDS Glossary Home The Clean Diet Nutrition & Immunity Immune Recovery Programs Nutrition Glossary The Aplastic Anaemia Trust Aplastic Anemia & MDS International Foundation

AA & MDS Glossary  

BAND CELL Noun: also a band, a stab, a stab cell, a band form. See stab cell for  definition and pictures.  

BASOPHIL Noun: One of the three granular white blood cells. It produces a  product which prevents the blood from clotting. 

This is an actual picture of a Basophil (in the centre) surrounded by  some red blood cells. The basophil is named after a basic dye that stains  it. Greek ­ básis = basic (from the basic dye that stains it) Greek ­ phîlos = lover  

B ­ CELL

Noun: An abbreviation for B Lymphocyte .   

BIOPSY Noun: The surgical removal of tissue from a living body for examination  and diagnosis. Also the medical examination of this tissue. Greek ­ bios = life Greek ­ ópsis = a viewing  

BLAST Noun: An immature cell. Greek ­ blastós = germ sprout   

BLOOD Noun: Blood is essential for life. Blood carries oxygen, nutrients,  hormones and chemicals to each of the sixty billion cells throughout the  body. It plays an essential part in protecting the body from infection.  Blood also helps the body remove waste and toxins. There are close to 30  trillion blood cells in an adult. Each cubic millimetre of blood contains  from 4 1/2 to 5 1/2 million red blood cells and an average total of 7,500  white blood cells.  All types of blood cells are produced by the bone marrow . The bone  marrow is the soft, spongy tissue found in the centre of the large bones  in the body. There are four main components of blood: red blood cells,  white blood cells, platelets, and liquid plasma . Since red and white 

blood cells are continually being destroyed, they body must continue to  produce new ones in the bone marrow. About 2 1/2 million new red  blood cells are created every second.  

BLOOD TEST Noun: An action where one or more samples of blood are taken in order  to determine blood levels, illness, tissue type, blood type or  abnormalities. The test result will have a number of abbreviations on it. I will outline  them here so you can understand what they are about. Percentages and  numbers are measured on the white blood cells so that by multiplying  the percentage by the total count we have an actual number of each type  of cell. WBC ­This measures the number of white blood cells in a very small  quantity of blood (a billionth of a litre). It's the White Blood Cell Count NE ­ is the neutrophil count or percentage. 50­60% is normal. Numbers  for these and the following measurements differ for men, women and  children. Those average figures can be found in booklets given to you by  the support group.  LY ­ is the lymphocyte count or percentage. 20­40% is normal MO ­ is the monocyte count or percentage. 2­9% is normal EO ­ is the eosinophil count or percentage. 1 ­ 4% is normal BA ­ is the basophil count or percentage. 0.5 ­ 2% is normal RBC ­ is the red blood count. 

HGB ­ is the haemoglobin count. It is considered a considerable  anaemia when the haemoglobin drops below 10 for an adult. HCT ­ is the haematocrit percentage of red blood cells in the sample.  MCV ­ is the mean (average) corpuscular volume. This measures the  average volume of red blood cells. Normal is 84 to 99 fl (a femto­litre is a  quadrillionth of a litre). Don't ask me how they can measure anything so  small! MCH ­ is the mean corpuscular haemoglobin which is a measurement  of the amount of haemoglobin in an average cell. Normal is 26 to 32 pg  (a picogram is a trillionth of a gram). MCHC ­ is the mean corpuscular haemoglobin concentration which is a  measurement of the average concentration of haemoglobin in red blood  cells. RDW ­ is the red blood cell distribution width. RET ­ is the reticulocyte number or percentage. Don't want too many  immature red blood cells. PLT ­ is the platelet count. A count below 50 can result in spontaneous  bleeding, and below 5, patients are at risk of severe life­threatening  haemorrhaging . MPV ­ is the mean (average) platelet volume which is a measurement of  the average volume of platelets in the sample.   

B LYMPHOCYTE Noun: The B cell. It is a type of lymphocyte that has matured in the bone  marrow and mainly deals with bacteria and viruses that have been 

encountered before. Unlike T­cells , they do not circulate in the blood.  Their home is in the Lymphatic System . When an invader is present, T­ cells or macrophages present the invaders antigen to the B lymphocyte  cell and it takes the invader into the tissues where it determines its exact  size and shape. It then makes an exactly fitting straitjacket called an  antibody that will fit that intruder and no other. Then it gets a  production line going to produce thousands more of these antibodies.  These move through the body and attach themselves to the micro­ organisms which make them harmless and held until the macrophages  or neutrophils come along to devour them. B­cells work with T­cells,  macrophages and neutrophils to destroy harmful substances which have  entered the body. B­cells can memorise the invader's antigen and  become a long lived memory B­cell. This results in a quicker response to  an infection. Each memory B­cell is specific to one particular antigen. English ­ B = bone + Latin ­ lympha = clear water + Greek ­ kytos = anything hollow.  

BMT Abbreviation: Bone Marrow Transplant  

BONE MARROW Noun: The growth and development of normal cells are carefully  controlled in the bone marrow to produce the correct numbers of each  type of blood cell to keep the body healthy. Although, there are many  different types of blood cells, all cells made in the bone marrow start as  a single kind of cell called a stem cell . Stem cells make up only a very  small proportion of the cells in the bone marrow.

This is a picture of normal bone marrow at medium magnification.  About one half of the marrow is filled with red blood cells, white blood  cells, platelets,and the cells which produce them. The large white cells  are fat cells called steatocytes . The bone marrow stores stem cells until the body needs a specific type  of mature blood cell. Then, by using the stem cells in reserve, the bone  marrow can rapidly produce many red cells, white cells or platelets. As  stem cells mature, their features become more and more distinct until  the stem cells develop into a specific type of blood cell. See  Haematopoiesis for a picture of the process.  

BONE MARROW BIOPSY Verb: In all patients with aplastic anaemia or myelodysplasia , a sample  of bone, and bone marrow will be required. This involves obtaining a  small amount of marrow from inside the bone with a needle and a  sample of the bone itself showing the structure of the bone marrow 

cavity (small holes in the bone where the bone marrow grows). This is  called a Bone Marrow Aspirate . The small piece of bone is called a bone  marrow trephine. The samples used are usually obtained from the back  of the hip bone. The procedure causes some discomfort but does not  take very long. The procedure is usually carried out with sedation as  well as local anaesthetic. It may be necessary to sample more than one  site in aplastic anaemia or myelodysplasia to confirm that there is no  other bone marrow disease present.  The main bone marrow finding which defines aplastic anaemia is that  the few blood producing cells which are present, appear normal. The  cells in aplastic anaemia do not show chromosome abnormalities. In myelodysplasia or myelofibrosis , the numbers of blood producing  cells are considerably reduced. The cells which are present in the bone  marrow in these diseases are very abnormal under the microscope.

These are some red blood cells from a patient with Myelodysplasia.  They are larger than normal and very irregular.  

BONE MARROW TRANSPLANT Verb: A procedure used to treat Aplastic Anaemia , Myelodysplasia ,  Acute Leukaemia and some rare birth disorders with varying success. 

Healthy bone marrow is taken from the donor and infused into the  bloodstream of the recipient: from here, it 'homes' in on the bone  marrow, where it will grow. There is only a one in four chance that a full  brother or sister will be a match. Bone marrow transplantation is a risky procedure but success rates as  high as 80% have been reported when the donor is a closely matched  brother or sister. See graft­versus­host disease for a full appraisal of the  risks. The other major risk is that the immunosuppressant drugs used to  control GVHD make the patient more susceptible to infections.  Patients with aplastic anaemia should be transplanted without the use  of irradiation. Rejection of the graft is prevented by using a drug called  cyclophosphamide often together with antibodies including  antilymphocyte globulin which immunosuppress the recipient. The risk  of graft failure, that is rejection of the bone marrow transplant, is greater  for patients with aplastic anaemia than for patients with leukaemia . There are some clear recommendations for treatment choices. Children,  adolescents and young adults with brothers or sisters who are matched  donors should be transplanted. Patients who have no brothers or sisters  who are able to donate should be treated with immunosuppressive  drugs. High risk patients, who have very low neutrophil counts, should  receive intensive supportive treatment of blood and platelets prior to  treatment. For older patients who have brothers or sisters who are able to donate  their bone marrow, the opinion is more divided. Some specialists  recommend transplantation for any patient below the age of 50 years. In  Europe, I am told they only will recommend it for patients below the  age of 40 years. Other experts have recommended an initial trial of  immunosuppressive drugs followed by a bone marrow transplant if the 

immunosuppressive drugs fail to work or if myelodysplasia or  leukaemia later develop. Patients who fall into this group should  discuss the choices carefully with their specialist before arriving at a  decision on treatment.  Any patient diagnosed with severe aplastic anaemia or myelodysplasia  should have rapid HLA tissue typing performed to identify possible  marrow donors. Blood transfusions from prospective marrow donors  should be avoided. I have found a 45 minute movie on bone marrow transplants. It p articularly deals with Haplo transplants . What is Sickle Cell Anaemia? This page will give you a brief guide to Sickle Cell Disorders and provide links to more detailed information for different groups of users Contents

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What is Sickle Cell Anaemia? • Other types of Sickle Cell Disorders (SCDs) • Who Gets SCDs? • Testing for SCDs • Testing unborn babies • What Sickle Cell Disorders are Not • More Detailed Information

The disorder affects the red blood cells which contain a special protein called haemoglobin (Hb for short). The function of haemoglobin is to carry oxygen from the lungs to all parts of the body. People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickleshaped. Normal red blood cells can bend and flex easily. Because of their shape, sickled red blood cells can't squeeze through small blood vessels as easily as the almost donut-shaped normal cells. This can lead to these small blood vessels

getting blocked which then stops the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs. Everyone has two copies of the gene for haemoglobin; one from their mother and one from their father. If one of these genes carries the instructions to make sickle haemoglobin (HbS) and the other carries the instructions to make normal haemoglobin (HbA) then the person has Sickle Cell Trait and is a carrier of the sickle haemoglobin gene. This means that this person has enough normal haemoglobin in their red blood cells to keep the cells flexible and they don't have the symptoms of the sickle cell disorders. They do however have to be careful when doing things where there is less oxygen than normal such as scuba diving, activities at high altitude and under general anaesthetics. If both copies of the haemoglobin gene carry instructions to make sickle haemoglobin then this will be the only type of haemoglobin they can make and sickled cells can occur. These people have Sickle Cell Anaemia and can suffer from anaemia and severe pain. These severe attacks are known as Crises. Over time Sickle Cell sufferers can experience damage to organs such as liver, kidney, lungs, heart and spleen. Death can be a result. Another problem is that red blood cells containing sickle haemoglobin do not live as long as the normal 120 days and this results in a chronic state of anaemia. In spite of this, a person with sickle cell disorder can attend school, college and work. People with sickle cell disorder need regular medical attention particularly before and after operations, dental extraction and during pregnancy. Many hospitals arrange follow-up appointments and it is advisable to discuss with the doctors questions concerning schooling, strenuous exercise, family planning, suitable types of employment and air travel. When a person is found to have a sickle cell disorder it is important that all members of the family be tested. They will not necessarily have sickle cell disorder but may be healthy carriers of a sickle cell trait. Top Other types of Sickle Cell Disorders (SCDs) There are also other different types of haemoglobin such as HbC and beta thalassaemia, that can combine with sickle haemoglobin to cause sickling disorders. When someone carries the gene for beta-thalassaemia they cannot make as much HbA as they should. If this is combined with the HbS gene then more of their total amount of haemoglobin is HbS and they can suffer from what is usually a milder form of sickle cell disorder than sickle cell anaemia. Top Who Gets SCDs? The different kinds of SCD and the different traits are found mainly in people whose families come from Africa, the Caribbean, the Eastern Mediterranean, Middle East and Asia.* In Britain SCD is most common in people of African and Caribbean descent (at least 1 in 10-40 have sickle cell trait and 1 in 60-200 have SCD). It is estimated there are over 6,000 adults and

children with SCD in Britain at present. There are other inherited conditions that mainly affect other groups, e.g. Cystic Fibrosis in Europeans, and Tay-Sachs disease in Jewish people. Top Testing for SCDs A special blood test (haemoglobin electrophoresis) can tell you whether you have a sickle cell disorder or are a healthy carrier, e.g. for sickle cell trait. Routine screening should take place for people in the groups listed during pregnancy and before anaesthesia, either at hospital or dental clinics. Tests can be arranged by your general practitioner or at our local sickle cell centre. (Contact us for an up-to-date list of centres in the UK.) After a blood test you may be told that you or your child is `sickle positive' or has `sickle cell'. It is important to ask whether this means sickle cell trait or a type of sickle cell disorder. Top Testing unborn babies It is now possible to test the unborn child to detect a haemoglobin disorder from as early as 11 weeks of pregnancy. For more information see the Prenatal Testing page and contact your local sickle cell centre, local obstetrician, haematologist or general practitioner. Top What Sickle Cell Disorders are Not People are often confused and disturbed by some of the incorrect information they have received about SCD. Sickle Cell Disorders are are are are

not not not not

leukaemia cancer white blood cells eating up red blood cells infectious - you cannot `catch it' as you would catch measles or a cold.

Sickle Cell Disorder is a condition which is inherited from both parents. This means that people are born with it, just as they are born with other characteristics such as eye colour, hair texture and height. Top More Detailed Information

Follow the links to get more information on different aspects on sickle cell disorders.

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Day to day care for children and adults with Sickle Cell Disorders Inheritance and Prenatal Testing Information for Employees and Employers Teaching resources Facts for Health Professionals

For further information contact: Sickle Cell Society 54 Station Road London, NW10 4UA UK

Tel 020 8961 7795 Fax 020 8961 8346 [email protected]