Hairy cell leukemia is a disease in which a type of white blood cell called the lymphocyte, present in the blood and bone marrow, becomes malignant and proliferates. It is called hairy cell leukemia because the cells have tiny hair-like projections when viewed under the microscope.
Description
Hairy cell leukemia (HCL) is a rare cancer. It was first described in 1958 as leukemic reticuloendotheliosis, erroneously referring to a red blood cell because researchers were unsure of the cell of origin. It became more easily identifiable in the 1970s. There are approximately 600 new cases diagnosed every year in the United States, making up about 2% of the adult cases of leukemia each year.
HCL is found in cells located in the blood. There are three types of cells found in the blood: the red blood cells that carry oxygen to all the parts of the body; the white blood cells that are responsible for fighting infection and protecting the body from diseases; and the platelets that help in the clotting of blood. Hairy cell leukemia affects a type of white blood cell called the lymphocyte. Lymphocytes are made in the bone marrow, spleen, lymph nodes, and other organs. It specifically affects B-lymphocytes, which mature in the bone marrow. However, extremely rare variants of HCL have been discovered developing from T-lymphocytes, which mature in the thymus.
When hairy cell leukemia develops, the white blood cells become abnormal both in the way they appear (by acquiring hairy projections) and in the way they act (by proliferating without the normal control mechanisms). Further, the cells tend to accumulate in the spleen, causing it to become enlarged. The cells may also collect in the bone marrow and prevent it from producing normal blood cells. As a result, there may not be enough normal white blood cells in the blood to fight infection.
Demographics
The median age at which people develop HCL is 52 years. Though it occurs in all ages, HCL more commonly develops in the older population. Men are four times more likely to develop HCL than women. There have been reports of familial aggregation of disease, with higher occurrences in Ashkenazi Jewish men. A potential genetic link is undergoing further investigation.
Causes and symptoms
The cause of hairy cell leukemia is not specifically known. However, exposure to radiation is a known cause of leukemia in general. Familial involvement is another theory, suggesting that there is a genetic component associated with this disease.
HCL is a chronic (slowly progressing) disease, and the patients may not show any symptoms for many years. As the disease advances, the patients may suffer from one or more of the following symptoms:
Pain and discomfort are caused by an enlarged spleen, which results from the accumulation of the abnormal hairy cells in the spleen. Blood tests may show abnormal counts of all the different types of cells. This happens because the cancerous cells invade the bone marrow as well and prevent it from producing normal blood cells. Because of the low white cell count in the blood, the patient may have frequent infections. Fever often accompanies the infections. The patient is most susceptible to bacterial infections, but infections of any kind are the major cause of death. The low red cell count may cause anemia, fatigue, and weakness, and the low platelet count may cause the person to bruise and bleed easily.
Diagnosis
When a patient suffers from the above symptoms, the doctor will palpate (examine with fingers) the abdomen and may order scans to see if the spleen is enlarged (splenomegaly). An enlarged spleen is present in 80% of patients. An enlarged liver is less common, but can occur.
If the spleen is enlarged, the doctor may order several blood tests. In these tests, the total numbers of each of the different types of blood cells (CBC) are reported. Sixty to eighty percent of patients suffer from pancytopenia, which is a dramatic reduction in the number of red blood cells, white blood cells, and platelets circulating in the blood.
If the blood tests are abnormal, the doctor may order a bone marrow aspiration and biopsy. In order to establish a diagnosis, hairy cells must be present in the bone marrow.
Treatment team
If the patient is seeing a primary care provider, the provider may perform the initial diagnostic tests. However, in order to diagnose and treat HCL comprehensively, the primary care provider will refer the patient to an oncologist (cancer specialist). Radiologists and pathologists will also be involved to read scans and examine tissue samples. Other specialists involved with the treatment of hairy cell leukemia will be nurses and dieticians who are available to explain side effects of treatment and offer suggestions on eating healthy meals that may help fight the side effects.
Clinical staging, treatments, and prognosis
When physicians perform blood tests, they will determine the level of hemoglobin (the oxygen-transporting molecule of red blood cells). Serum hemoglobin levels and the size of the spleen, which can be measured on exam and by using an x ray, are proposed criteria for determining the stage of HCL. The following are the three proposed stages and their criteria:
Stage I: Hemoglobin greater than 12 g/dL (1 g = approximately 0.02 pint and 1 dL = approximately 0.33 ounce) and spleen less than or equal to 10 cm (3.9 inches).
Stage II: Hemoglobin between 8.5 and 12 g/dL and spleen greater than 10 cm (3.9 inches).
Stage III: Hemoglobin less than 8.5 g/dL and spleen greater than 10 cm (3.9 inches).
Since there is generally no accepted staging system, another method for evaluating the progression of HCL is to group patients into two categories: untreated HCL and progressive HCL, in which hairy cells are present after therapy has been administered.
Some people with hairy cell leukemia have very few or no symptoms at all, and it is reasonable to expect that 10% of patients may not need any treatment. However, if the patient is symptomatic and needs intervention, HCL is especially responsive to treatment.
There are three main courses of treatment: chemotherapy, splenectomy (surgical removal of the spleen), and immunotherapy. Once a patient meets treatment criteria, purine analogues, particularly the drugs, pentostatin and cladribine, are the first-line therapy. Pentostatin is administered at 5mg/m2 for two days every other week until total remission is achieved. Patients may experience side effects such as fever, nausea and vomiting, photosensitivity, and keratoconjuctivitis. However, follow-up studies estimate a relapse-free survival rate at 76%. Cladribine (2-CdA) taken at 0.1mg/kg/day for seven days also has an impressive response. Eighty-six percent of patients experience complete remission after treatment, while 16% experience partial remission. Fever is the principal side effect of 2-CdA.
Biological therapy or immunologic therapy, where the body's own immune cells are used to fight cancer, is also being investigated in clinical trials for hairy cell leukemia. A substance called interferon that is produced by the white blood cells of the body was the first systemic treatment that showed consistent results in fighting HCL. The FDA approved interferon-alpha (INF-alpha) to fight HCL. The mechanism by which INF-alpha works is not clearly understood. However, it is known that interferons stimulate the body's natural killer cells that are suppressed during HCL. The standard dosage is 2 MU/m2 three times a week for 12 months. Side effects include fever, myalgia, malaise, rashes, and gastrointestinal complaints.
If the spleen is enlarged, it may be removed in a surgical procedure known as splenectomy. This usually causes a remission of the disease. However, 50% of patients that undergo splenectomy require some type of systemic treatment such as chemotherapy or immunotherapy. Splenectomy is not the most widely used course of treatment as it was many years ago. Although the spleen is not an indispensable organ, it is responsible for helping the body fight infection. Therefore, other therapies are preferred in order to salvage the spleen and its functions.
Most patients have excellent prognosis and can expect to live 10 years or longer. The disease may remain silent for years with treatment. Continual follow-up is necessary to monitor the patient for relapse and determine true cure rates.
Alternative and complementary therapies
Many individuals choose to supplement traditional therapy with complementary methods. Often, these methods improve the tolerance of side effects and symptoms as well as enrich the quality of life. The American Cancer Society recommends that patients talk to their doctor to ensure that the methods they are using are safely supplementing traditional therapy. Some complementary treatments include the following:
The treatment and the disease interfere with the patient's ability to produce red blood cells, white blood
cells, and platelets, causing the patient to be vulnerable to anemia and life-threatening infection and bleeding. Transfusions can be given to patients in order to increase the number of red blood cells and platelets in the blood. In addition, colony-stimulating factors are being studied. These increase the number of the patient's own white blood cells.
Nausea and vomiting can result from chemotherapy and are often controlled by prescription drugs called antiemetics. Patients can also curb nausea and vomiting by eating slowly and avoiding large meals. Drinking water an hour before meals and staying away from foods that are sweet or fried is also helpful. Many times, chemotherapy is handled better if the patient is eating
well. Nurses and dieticians can aid patients in choosing healthful foods to incorporate into their diet.
Patients can fight anemia and fatigue by getting plenty of rest and minimizing strenuous activities. A well-balanced diet can also counter anemia and fatigue.
Although physicians will do everything possible to keep a patient's blood count high, there are precautions that can be taken by patients in order to reduce their risk of infection. Patients should regularly wash their hands, especially before and after eating meals and after using the restroom. Patients should avoid individuals who are contagious with colds, the flu, or the chicken pox. It is also helpful if patients do not cut themselves or do anything to expose deeper layers of the skin where bacteria can contaminate and cause infection. Finally, patients should avoid large crowds.
Clinical trials
Clinical trials are being performed to improve the effectiveness of treatment and to minimize the side effects. Patients may choose to volunteer for a clinical trial if they do not respond to standard therapy or if they want to reduce side effects. Clinical trials for the treatment of HCL involving purine analogues were being researched in 2001. Clinical trials are being performed all over the United States, and patients should discuss options with their doctors or contact the Cancer Information Service at (800) 4-CANCER (800-422-6237).
In many cases, insurance companies will not cover procedures that are part of clinical trials. Patients should talk with their doctor and insurance company to determine which procedures are covered.
Prevention
Since the cause for the disease is unknown and there are no specific risk factors, there is no known prevention.
Special concerns
Cancer treatments and their side effects can take a physical and psychological toll on cancer patients and their families. To deal with the psychological impact, there are many different support groups and psychotherapists that can help. Psychiatrists can prescribe medication to help with depression. Support groups can encourage and strengthen the psyche by relating to one another through shared experiences and success stories. Relying on faith practices is also beneficial for cancer patients to deal with their condition. Patients should discuss all options with their physician to determine what is available to them.
—A condition in which there is low iron in the blood due to a deficiency of red blood cells.
Bone marrow
—The spongy tissue inside the large bones in the body that is responsible for making the red blood cells, white blood cells, and platelets.
Bone marrow aspiration and biopsy
—A procedure in which a needle is inserted into the large bones of the hip or spine and a small piece of marrow is removed for microscopic examination.
Immunotherapy
—A mode of cancer treatment in which the immune system is stimulated to fight the cancer.
Leukemia
—A disease in which the cells that constitute the blood become cancerous or abnormal.
Lymph nodes
—Oval-shaped organs that are the size of peas, located throughout the body, and contain clusters of cells called lymphocytes. They filter out and destroy bacteria, foreign particles, and cancerous cells from the blood.
Malignant
—Cells that have the ability to invade locally, cause destruction of surrounding tissue, and travel to other sites in the body.
