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Leukemia - B-cell Prolymphocytic Leukemia and Hairy Cell Leukemia

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What is Leukemia - B-cell Prolymphocytic Leukemia and Hairy Cell Leukemia ?

About leukemia

Leukemia is a cancer of the blood cells. Leukemia begins when normal blood cells change and grow uncontrollably. Blood cells are made in the bone marrow, the spongy tissue inside the larger bones in the body. There are different types of blood cells, including red blood cells that carry oxygen throughout the body, white cells that fight infection, and platelets that help the blood to clot. Types of leukemia are named after the specific blood cell that becomes cancerous, such as the lymphoid cells (white blood cells of the immune system) or the myeloid cells (cells of the bone marrow which develop into cells that fight bacterial infections). There are four main types of leukemia in adults:

Acute lymphocytic leukemia (ALL)

Chronic lymphocytic leukemia (CLL)

Acute myeloid leukemia (AML)

Chronic myeloid leukemia (CML)

About PLL and HCL

There are other, less common types of leukemia, but they are generally subcategories of one of the four main categories. This section focuses on prolymphocytic leukemia (PLL) and hairy cell leukemia (HCL), both of which are chronic B-cell leukemias. B cells are a specific type of lymphocyte that normally make antibodies for the immune system.

In PLL, many immature lymphocytes, or prolymphocytes, are found in the blood. This type of leukemia may occur by itself, together with CLL, or CLL may turn into into PLL.  PLL tends to worsen more quickly than CLL. 

HCL is a slow-growing form of leukemia. It is called “hairy cell” because the abnormal lymphocytes have projections that look like hair when seen under a microscope. As these cells multiply, they build up in the bone marrow, blood, and spleen. Because these lymphocytes are abnormal, they do not work normally to fight disease and infection, and eventually may crowd out the normal cells. Treatment is often very effective for HCL.

Symptoms & Signs

People with B-cell leukemia may experience the following symptoms or signs. Sometimes, people with B-cell leukemia do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Enlarged lymph nodes in PLL. In HCL, hairy cells build up more often in the spleen and bone marrow and may not cause noticeable lymph node swelling.

Tendency to bruise easily

Slow wound healing

Weight loss

Unexplained fatigue

Persistent, unexplained abdominal pain on the left side, due to an enlarged spleen

Frequent infections

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptoms(s) and how often.

If leukemia is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Risk Factors

risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

In general, leukemia can be caused by a genetic mutation (change) that is inherited (passed from generation to generation within a family) or environmental factors, such as smoking, or chemical or radiation exposure. However, most often the cause of leukemia is not known.

The following factors may raise a person’s risk of developing PLL or HCL:

Age. HCL occurs most often in people between ages 40 and 70.

Gender. Men are five times more likely to develop HCL than women.

Diagnosis

Doctors use many blood and bone marrow tests to diagnose leukemia and to find out the extent to which it may have spread. Although a patient’s signs and symptoms may cause a doctor to suspect leukemia, it is diagnosed only by blood and bone marrow tests. Some tests may also determine which treatments may be the most effective. Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Severity of symptoms

Previous test results

The following tests may be used to diagnose PLL and HCL:

Blood tests. The diagnosis of PLL or HCL begins with a blood test, called a complete blood count (CBC), that measures the numbers of different types of cells in a person's blood. If the blood contains many white blood cells, B-cell leukemia may be suspected. However, patients with HCL often have a very low level of white blood cells.

Bone marrow biopsy and aspiration. These two procedures are similar and often done at the same time. Bone marrow has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease) to determine the number and type of abnormal cells. A common site for the bone marrow biopsy and aspiration is the pelvic bone, which is located in the lower back by the hip. The skin in that area is usually numbed with medication beforehand, and other types of anesthesia (medication to block the awareness of pain) may be used.

Molecular testing. Your doctor may recommend running laboratory tests on the leukemia cells to identify specific genes, proteins, and other factors unique to the leukemia. Immunophenotyping is the examination of antigens, a specific type of protein, on the surface of the leukemia cells. Immunophenotyping allows the doctor to confirm the exact type of leukemia. Cytogenetics is the examination of the leukemia cells for chromosome (long strands of genes) abnormalities. It helps the doctor confirm the diagnosis and may help to determine the person’s prognosis (chance of recovery). Results of these tests will also help decide whether your treatment options include a type of treatment called targeted therapy.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail. A CT scan also shows enlarged lymph nodes or a swollen spleen.

Stages

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis. There are different stage descriptions for different types of cancer.

Unlike most solid tumors, HCL and PLL do not have a formal staging system.  Talk with your doctor for details on how your specific disease will be evaluated.

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