Chronic leukaemia

What is chronic leukaemia?

Leukaemia literally means ‘many white cells in the blood'.

The white cells are part of the body’s immune system, and there are several sub-groups of white cells that have different sorts of roles in recognising and dealing with ‘invaders’ such as bacteria and viruses as well as other types of foreign protein.

All of the blood cells originate in the bone marrow and leukaemia (blood cancer) is a disease where the bone marrow produces large numbers of abnormal white cells. This means that the normal marrow is pushed into smaller and smaller areas. This results in fewer normal cells being produced and gives rise to some of the symptoms.

There are many types of leukaemia, each of which is classified according to the exact cell type affected by the disease.

Chronic leukaemia is a slowly progressive form of leukaemia and tends to involve more mature cell types. It may not need treatment immediately, but if treatment is required it is usually chemotherapy, given in the form of tablets.

The cause of leukaemia is not known.


What are the symptoms of chronic leukaemia?

It is possible to have chronic leukaemia for months or even years without knowing it.

The symptoms are varied, but many people notice:

* tiredness (due to anaemia)
* bruising easily (often without having had any blow or fall)
* repeated infections
* enlarged lymph glands
* weight loss
* night sweats
* fever.


What is chronic lymphocytic leukaemia?
Chronic lymphocytic leukaemia (CLL) is the commonest type of leukaemia with 3000 to 4000 new cases diagnosed each year in the UK. It is a form of chronic leukaemia characterised by an increased number of lymphocytes, which make up one of the main sub-groups of white cells in the blood. Despite their increased numbers these lymphocytes lack the normal ability of responding to infection by the production of antibodies, so compromising the immune system of the affected person.

CLL is rarely found in people under the age of 40 - the peak age is 65. It is twice as common in men than in women. There are no obvious causes known for CLL.


What are the symptoms of chronic lymphocytic leukaemia?
In addition to the symptoms mentioned above which are common to all forms of leukaemia, specific signs of the disease are:

* painless enlargement of the lymph glands especially in the neck, armpits and groin.
* lymph glands in deeper parts of the body may need special scans for diagnosis.
* sometimes an enlarged spleen (located in the left upper quadrant of the abdomen) may cause discomfort or pain.


How is chronic lymphocytic leukaemia diagnosed?
Often the condition is diagnosed by chance when blood tests are being performed for other reasons.

Although a blood test may give doctors the diagnosis, a bone marrow test is usually done to confirm the diagnosis. Special tests are performed on these samples to help classify the leukaemia as this will influence the kind of treatment required.

Scans and X-rays may also be performed in order to help doctors decide on the best treatment.


How is chronic lymphocytic leukaemia treated?
Treatment is not always required and the patient may just be followed up as an outpatient on a regular basis, sometimes for many years, with no need for further action.

Older people with early stage CLL have a normal life expectancy. Treatment in the form of chemotherapy will be required for those who are unwell or who have many enlarged lymph glands, or who become significantly anaemic.

Chemotherapy is usually given in the form of tablets (usually a medicine called chlorambucil (Leukeran)). Other chemotherapy drugs such as fludarabine (Fludara) (may be used in late stage disease. General bone marrow production of blood cells can occur in more advanced CLL (bone marrow failure) in which steroid treatment with prednisolone (eg Deltacortril) usually allows the bone marrow to recover. Milder degrees of bone marrow failure might adequately be controlled by periodic blood transfusion.

X-ray treatment (radiotherapy) can be given locally, to swollen lymph nodes, or in small repeated doses to the whole body. Infections are more common in people with CLL and need to be diagnosed early and treated vigorously.

Sometimes the spleen, which is also part of the body’s immune system swells up so much in CLL that it gives rise to pain, or it causes a type of anaemia to develop in which the red cells of the blood (oxygen-carrying cells) become fragile, leading to further anaemia. These problems may justify the surgical removal of the spleen.

Bone marrow transplantation may be considered for those patients who are less than 45 years of age and who have an aggressive form of the disease.

Glycoprotein Storage Diseases

Glycoprotein & Related Storage Diseases are very rare, progressive, largely untreatable metabolic enzymatic defects inherited by children from both parents. The worldwide incidence for these diseases as a group has not yet been determined accurately, though they can certainly be classified as ultra-rare. The course of these disorders affects multiple systems of the body and clinical symptoms may vary patient-to-patient, and even among siblings. For most children the implications are eventual loss of mental and physical functions, and a premature death.

As the name implies, Glycoproteins are complex compounds composed of a protein and a carbohydrate. There are many different types of glycoproteins and they are found in abundance in all kinds of cells, including those of the brain. Glycoproteins play many roles in cells; some of these are well known, but others have yet to be discovered. Known roles include acting as an agent for communication between cells and assisting in maintaining the cell’s structure. The carbohydrate portion of glycoproteins is usually made of combinations of sugar molecules such as glucose, galactose, mannose and fucose, which are collectively known as oligosaccharides.

Normal functioning of the cell is characterized by the continual degradation of glycoproteins by enzymes within the lysosomes, which are membrane-bound compartments in the cell and essentially the cell’s recycling center. Specific enzymes are needed along each “step” of the recycling process in order that the continual, complete breakdown of these chains of glycoproteins is carried out. Any malfunction along the way results in the premature termination of the process with a resulting accumulation of the undegraded material within the lysosome. Today we recognize six different types of diseases caused by a defect in glycoprotein degradation: Alpha-Mannosidosis, Aspartylglucosaminuria, Beta-Mannosidosis, Fucosidosis, Galactosialidosis, Schindler Disease and Sialidosis. Two other related Glycoprotein Storage Diseases are I-Cell Disease (Mucolipidosis II) and Pseudo-Hurler Polydystrophy (Mucolipidosis III). These disorders are caused by trafficking errors that limit normal targeting of digestive enzymes, which are themselves glycoproteins, to the lysosomes and prevent the lysosome from functioning normally.

These diseases are a subset of a larger group of disorders known as Lysosomal Storage Diseases, which consist of over 40 autosomal recessive inherited metabolic diseases.

Inheritance
Glycoprotein & Related Storage Diseases are autosomal recessive conditions, meaning that a defective trait or mutation passed on from both parents is required for the disease to be expressed. Every person carries two copies of a gene, one of which they pass on to their offspring (the other provided by the second parent). In many cases, we all have a mutation or defect in some of our genes, but do not show signs of the defect because the other gene is able to allow us to function “normally.” In autosomal recessive diseases, the condition occurs when both copies of a gene are defective.

Since each parent donates a single gene copy to each offspring, there are four possible inheritance patterns for diseases to occur.


Since most people can live apparently normal lives with one healthy half of a gene pair, recessive gene disorders are relatively rare. However, as shown above, 50 % of people inheriting an unexpressed, or recessive, gene defect are known as carriers. Carriers have the ability to continue a family’s mutation to the next generation. Over time, though, the mutation will become diluted unless a carrier mates with another carrier.

Two carrier parents have a 25% chance of giving birth to a child with an autosomal recessive disease. Because these odds occur for each pregnancy, it is possible, though not likely, that a family may have more than one child with a genetic disease. There is a greater likelihood, 50%, that offspring of two such parents will be a carrier and continue to pass on the defect to future generations. Lastly, a 25% chance exists that a child born to two carrier parents will be completely healthy. Such a child inherits two good gene copies and, thus, will not pass the gene defect to its heirs.

For couples who have a known history of the same recessive genetic disease in their families, carrier testing is a possibility to assist with family planning. However, testing for a genetic defect without such prior knowledge or history is not possible or recommended for most autosomal recessive diseases.

SHE Wu Yue Tian (PLAY) 五月天

One of moi favourite songs from SHE's PLAY album

SHE zhong guo hua music video (PLAY) 中國話

one of moi favourite songs from SHE's latest PLAY Album
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