Sezary Syndrome: Causes, Symptoms, and Treatment

Sezary syndrome is a condition that is known to be more aggressive compared to any form of cancer. This syndrome is also known as cutaneous T-cell lymphoma. Sezary cells themselves are certain types of white blood cells with which one’s body will find cancerous cells in the lymph nodes, skin, and blood. To differentiate it with blood cancer, you must know symptoms of blood cancer.

Sezary Syndrome: Causes, Symptoms, and Treatment

These cancerous cells have the potential to spread to other organs and although this condition is uncommon, this syndrome is worth watching out for before it actually gets worse in a one’s body. 

That is why we need to recognize the causes, symptoms and how to treat them first.

Causes and Stages of Sezary Syndrome

It is not yet clear what factors cause Sezary’s syndrome to occur, but from many cases that has already occurred shows that the sufferer has a chromosomal abnormality in the DNA of cancer cells but is not found in the healthy cells. Chromosomal abnormalities like this do not include inherited defects but changes that occur have the potential to last a lifetime sufferers.

The most common abnormality in sufferers of this syndrome is the loss of DNA from chromosomes 10 and 17. Not only that, there are also abnormalities in which DNA actually increases in chromosomes 8 and 18 so the cause is uncertain. Even so, it is not yet clear whether the disorder is a cancer-causing factor.

This syndrome is not an inherited abnormalities or diseases because it is known that the inheritance pattern has not been fully determined. This is because someone whose family does not have a history of Sezary’s syndrome seems to be able to suffer this syndrome. So, this disease is not a hereditary disease; at least that’s what is found in most cases of Sezary syndrome until now.

Like cancer, which has stages of development and spreading out to the body through cells in the body of the sufferer, so does Sezary’s syndrome.

  • 1A – Red patches or rashes covering the skin less than 10%.
  • 1B – The skin covered by a reddish rash is more than 10%.
  • 2A – Skin that has more rashes and lymph nodes has begun to be bigger but not cancerous.
  • 2B – Tumors over 1 cm in size begin to appear on the skin. At this stage, lymph nodes are enlarged but there is no cancer.
  • 3A – Most of the skin has been exposed to or covered by a reddish rash which may also have been accompanied by tumors and plaques. Enlarged lymph nodes remain without cancer. Blood has the potential to contain or not contain Sezary cells.
  • 3B – Most of the skin has lesions and lymph nodes still have the potential to be larger even though they are uncertain. The number of Sezary cells in the blood is low.
  • 4A (1) – The surface of the skin has been partially covered by lesions and the number of Sezary cells begins to increase in the blood.
  • 4A (2) – The surface of the skin is partially covered by the lesion and the cells in the enlarged lymph nodes appear abnormal after being examined under a microscope. There is a potential for young cells to be present or not in the blood.
  • 4B – The surface of the skin partially covered by lesions and enlarged lymph nodes begin to show abnormalities, although not always. Sezary cells may already be in the blood and lymphoma cells have spread to other tissues and organs.

Symptoms of Sezary Syndrome

Sezary’s syndrome is characterized primarily by erythroderma, which is an itchy reddish rash that attacks the body. Almost 80% of the patient’s body will be covered in rash. If this happen, you might want to try ways on how to cure rash. Of course apart from these symptoms, there are other signs that accompany it.

  • Swollen lymph glands.
  • The skin swells.
  • Hair loss.
  • Disturbed body temperature regulation.
  • Skin tumors and plaques.
  • Abnormalities in the nails of the hands and feet.
  • Thickening of the skin, especially in the soles of the feet and hands.
  • Enlargement of the spleen.
  • Disorders of the lungs.
  • Disorders of the liver.
  • Indigestion.
  • The lower eyelid flips outwards.
  • Peeling skin.
  • The skin hurts.
  • Cold sensation in the body and sufferers will shiver.
  • Peripheral adenopathy.

One thing you have to be cautious for is that this disease can increase the risk of developing cancer in the body because this syndrome itself has an aggressive form of cancer.

Diagnosis and Treatment of Sezary Syndrome

Early diagnosis usually requires a physical examination by a doctor and this procedure needs to be carried out thoroughly. The doctor will then proceed with evaluating the patient’s medical history. In order for doctors to be more helpful in detecting and making the right diagnosis, some of the diagnostic tests below need to be taken by the patient.

  • Bone marrow biopsy.
  • Immunophenotyping
  • Immunogenotyping
  • Peripheral blood smear.
  • Flow cytometry test.
  • Blood test.
  • MRI Examination.
  • PET scan.
  • CT Scan..

After being diagnosed, and a positive result is obtained, sufferer might experience symptoms of Sezary syndrome, and doctors will provide treatment solutions for patients. Proper treatment is determined by several factors, such as age, the stage of the syndrome, and the presence of other health problems.

  • Radiation Therapy – In treating Sezary syndrome, it is necessary to have radiation therapy which aims to destroy cancer cells that is very possible to develop. Special rays will be sent to the area targeted at the body where pain and other symptoms appear, and make them all disappear. This method sometimes bring out side effects, so make sure you’re well aware of ways to prevent radiation therapy side effects.
  • PUVA/Psoralen and UVA – This method of treatment is presumably recommended by the doctor by injecting it into the patient’s veins. These drugs with the term psoralen will then be activated when exposed to UVA rays directed at the patient’s skin and from this procedure the cancer cells are destroyed without endangering too much healthy tissue.
  • Chemotherapy – Treatments using chemotherapy drugs are usually given to patients so that cancer cells are completely eradicated and the spread can be stopped. Even so, consult everything first to avoid any side effects of chemotherapy, and gain more knowledge about foods to avoid after chemotherapy session and foods to avoid during chemotherapy session.
  • Photochemistry – When the patient has received certain drugs, a number of blood cells will be removed from the body. Treatment is done through UVA light.
  • Immunotherapy – Doctors are also likely to provide immunotherapy as a treatment where there will be drugs such as interferon which are used to program the patient’s immune system as cancer fighters.

Sezary syndrome is one of many serious disorders if you see from all the signs as well as possible causes. Presumably if symptoms appear, you should immediately check yourself to your doctor to avoid it being spread, be prevented, and also does not lead to serious other complications.

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