hairy cell leukemia (redirected from Hairy cell leukemias)

Hairy Cell Leukemia 

Definition

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.

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:

• weakness
• fatigue
• recurrent infections
• fever
• anemia
• bruising
• pain or discomfort in the abdominal area
• weight loss (uncommon)
• night sweats (uncommon)

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 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

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, 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 immunotherapy, 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 interferon stimulates 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 treatment

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:

• yoga
• meditation
• religious practices and prayer
• music therapy
• art therapy
• massage therapy
• aromatherapy

Prevention

Since the cause for the disease is unknown and there are no specific risk factors, there is no known prevention.

Key terms

Anemia — 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.

Keratoconjunctivitis — Inflammation of the conjunctiva and cornea of the eye.

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 the 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.

Spleen — An organ that lies next to the stomach. Its function is to remove the worn-out blood cells and foreign materials from the blood stream.

Splenectomy — A surgical procedure that involves the surgical removal of the spleen.

Resources

Books

Bast, Robert C. Cancer Medicine. Lewiston, NY:B.C. Decker Inc., 2000.

Haskell, Charles M. Cancer Treatment. 5th ed. Philadephia: W.B. Saunders Company, 2001.

Organizations

American Cancer Society. 1599 Clifton Road, N.E. Atlanta, Georgia 30329. (800) 227-2345. http://www.cancer.org.

Cancer Research Institute (National Headquarters). 681 Fifth Avenue, New York, N.Y. 10022. (800) 992-2623. http://www.cancerresearch.org.

Hairy Cell Leukemia Research Foundation. 2345 County Farm Lane, Schaumburg, IL 60194. (800) 693-6173.

Leukemia Society of America, Inc. 600 Third Ave, New York, NY 10016. (800) 955-4572.

National Cancer Institute. 9000 Rockville Pike, Building 31, Room 10A16, Bethesda, Maryland, 20892. (800) 422-6237. http://wwwicic.nci.nih.gov.

Oncolink. University of Pennsylvania Cancer Center. http://cancer.med.upenn.edu.

Other

"Coping With Side Effects." National Cancer Institute. July 2, 2001. http://cancernet.nci.nih.gov/chemotherapy/chemoside.html.

NCI/PDQ Patient Statement, "Hairy cell leukemia." National Cancer Institute, 2001.

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leukemia /leu·ke·mia/ (loo-ke´me-ah) a progressive, malignant disease of the blood-forming organs, marked by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow.leuke´mic

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acute leukemia  leukemia in which the involved cell line shows little or no differentiation, usually consisting of blast cells; it comprises two types, acute lymphocytic leukemia and acute myelogenous leukemia.

acute granulocytic leukemia  acute myelogenous l.

acute lymphoblastic leukemia  (ALL) one of the two major categories of acute leukemia, characterized by anemia, fatigue, weight loss, easy bruising, thrombocytopenia, granulocytopenia with bacterial infections, bone pain, lymphadenopathy, hepatosplenomegaly, and sometimes spread to the central nervous system. It is subclassified on the basis of the surface antigens expressed, e.g., B-cell type, T-cell type.

acute lymphocytic leukemia  acute lymphoblastic l.

acute megakaryoblastic leukemia , acute megakaryocytic leukemia a form of acute myelogenous leukemia in which megakaryocytes are predominant and platelets are increased in the blood.

acute monocytic leukemia  an uncommon form of acute myelogenous leukemia in which the predominating cells are monocytes.

acute myeloblastic leukemia 

1. a common type of acute myelogenous leukemia in which myeloblasts predominate; it is divided into two types on the basis of degree of cell differentiation.

2. acute myelogenous l.

acute myelocytic leukemia  acute myelogenous l.

acute myelogenous leukemia  (AML) one of the two major categories of acute leukemia, with symptoms including anemia, fatigue, weight loss, easy bruising, thrombocytopenia, and granulocytopenia.

acute myeloid leukemia 

1. acute myeloblastic l. (1).

2. acute myelogenous l.

acute myelomonocytic leukemia  a common type of acute myelogenous leukemia, with both malignant monocytes and monoblasts.

acute nonlymphocytic leukemia  acute myelogenous l.

acute promyelocytic leukemia  acute myelogenous leukemia in which more than half the cells are malignant promyelocytes.

acute undifferentiated leukemia  (AUL) acute myelogenous leukemia in which the predominating cell is so immature it cannot be classified.

adult T-cell leukemia/lymphoma  (ATL) an adult-onset, subacute or chronic malignancy of mature T lymphocytes, believed to be caused by human lymphotropic virus type I.

aleukemic leukemia  a form in which the total white blood cell count in the peripheral blood is not elevated; it may be lymphocytic, monocytic, or myelogenous.

basophilic leukemia  leukemia in which the basophilic leukocytes predominate.

chronic leukemia  leukemia in which the involved cell line is well differentiated, usually B lymphocytes, but immunologically incompetent.

chronic granulocytic leukemia  chronic leukemia of the myelogenous type, usually associated with a specific chromosomal abnormality and occurring in adulthood.

chronic lymphocytic leukemia  (CLL) chronic leukemia of the lymphoblastic type, characterized by lymphadenopathy, fatigue, renal involvement, and pulmonary leukemic infiltrates.

chronic myelocytic leukemia , chronic myelogenous leukemia, chronic myeloid leukemia chronic granulocytic l.

chronic myelomonocytic leukemia  a chronic, slowly progressing form characterized by malignant monocytes and myeloblasts, splenomegaly, and thrombocytopenia.

leukemia cu´tis  a cutaneous manifestation of leukemia resulting from infiltration of the skin by malignant leukocytes.

eosinophilic leukemia  a form in which eosinophils are the predominating cells.

granulocytic leukemia  myelogenous l.

hairy cell leukemia  chronic leukemia marked by splenomegaly and an abundance of large, mononuclear abnormal cells with numerous irregular cytoplasmic projections that give them a flagellated or hairy appearance in the bone marrow, spleen, liver, and peripheral blood.

histiocytic leukemia  acute monocytic l.

lymphatic leukemia , lymphoblastic leukemia, lymphocytic leukemia a form associated with hyperplasia and overactivity of the lymphoid tissue, with increased levels of circulating malignant lymphocytes or lymphoblasts.

lymphogenous leukemia , lymphoid leukemia lymphatic l.

lymphosarcoma cell leukemia  (B-cell type) acute lymphoblastic l.

mast cell leukemia  a rare form marked by overwhelming numbers of tissue mast cells in the peripheral blood.

megakaryoblastic leukemia  acute megakaryocytic l.

megakaryocytic leukemia 

1. acute megakaryocytic l.

2. hemorrhagic thrombocythemia.

micromyeloblastic leukemia  a form of myelogenous leukemia in which the immature nucleoli-containing cells are small and similar to lymphocytes.

monocytic leukemia  acute monocytic l.

myeloblastic leukemia 

1. myelogenous l.

2. acute myeloblastic l.

myelocytic leukemia , myelogenous leukemia, myeloid granulocytic leukemia a form arising from myeloid tissue in which the granular polymorphonuclear leukocytes and their precursors predominate. See also acute myelogenous l. and chronic granulocytic l.

myelomonocytic leukemia  acute myelomonocytic l.

plasma cell leukemia , plasmacytic leukemia a form in which the predominating cell in the peripheral blood is the plasma cell.

promyelocytic leukemia  acute promyelocytic l.

Rieder cell leukemia  a form of acute myelogenous leukemia in which the blood contains asynchronously developed cells with immature cytoplasm and a lobulated, relatively more mature nucleus.

stem cell leukemia  acute undifferentiated l.

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hairy cell leukemia

n.

A lymphocytic leukemia, usually originating with B cells, and characterized by splenomegaly and cells with a ciliated appearance in the spleen, bone marrow, liver, and blood. Also called leukemic reticuloendotheliosis.

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hairy

characterized by a covering of hairs.

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hairy angels' trumpet

daturametel.

hairy caltrop

kallstroemiahirsutissima.

hairy caterpillars

elongated mucosal erosions occur in mouths of horses grazing pasture infested with hairy caterpillars. See also erosive stomatitis.

hairy cell leukemia

a condition of humans, of which a counterpart is seen in cats. Previous name was reticuloendotheliosis.

hairy gousiektebossie

pachystigmapygmaeum.

hairy millet

see panicumeffusum.

hairy panic grass

see panicumeffusum.

hairy shakers

see border disease.

hairy thornapple

daturametel.

hairy vetch

see viciavillosa.

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hairy cell leukemia

Leukemic reticuloendotheliosis, tricholeukocytic leukemia
A low-grade B-cell leukemia comprising 2% of adult leukemia, commonly affecting ♂–♂:♀ ratio, 4:1, age 50-55, leading to progressive pancytopenia Clinical Insidious onset, weight loss, bruising, abdominal fullness due to splenomegaly, pancytopenia with normocytic, normochromic anemia, rarely aplastic anemia due to infiltration of BM, spleen, lymph nodes; 10% have platelet counts of < 20 x 109/L–US: < 20,000/mm3, 20% have thrombocytosis; 1-80% of nucleated RBCs are hairy Lab ↑ Acid phosphatase, especially isoenzyme 5–which is ↑ in bone metastases and in Gaucher's disease Management Purine analogues 2'-deoxycoformycin–pentostatin and 2-chlorodeoxyadenosine–cladribine, a purine nucleoside used in low-grade lymphoproliferations–eg, CLL and NHL is more effective than IFN-α Cause of death Infections, gram-negative bacteria, atypical mycobacteria, fungi. See Dry tap.