Online Medicine Resource: Hairy Cell Leukaemia

Hairy cell leukaemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).
Gender and age may affect the risk of developing hairy cell leukaemia.
Possible signs of hairy cell leukaemia include tiredness, infections, and pain below the ribs.
Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukaemia.
Certain factors affect treatment options and prognosis (chance of recovery).

Hairy cell leukaemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

Hairy cell leukaemia is a cancer of the blood and bone marrow. This rare type of leukaemia gets worse slowly or not at all. The disease is called hairy cell leukaemia because the leukaemia cells look "hairy" when viewed under a microscope.

Normally, the bone marrow produces stem cells (immature cells) that develop into mature blood cells. There are 3 types of mature blood cells:

Red blood cells that carry oxygen and other materials to all tissues of the body.
White blood cells that fight infection and disease.
Platelets that help prevent bleeding by causing blood clots to form.

In hairy cell leukaemia, too many stem cells develop into a type of white blood cell called lymphocytes. These lymphocytes may also be called leukaemic cells. There are 3 types of lymphocytes.

B lymphocytes that make antibodies to help fight infection.
T lymphocytes that help B lymphocytes make antibodies to help fight infection.
Natural killer cells that attack cancer cells and viruses.

In hairy cell leukaemia, the B lymphocytes do not work normally. Also, as the number of lymphocytes increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukaemia cells may collect in the spleen and cause it to swell.

This summary is about hairy cell leukaemia. Refer to the following summaries for information about other types of leukaemia:

Adult Acute Lymphoblastic Leukaemia Treatment.
Childhood Acute Lymphoblastic Leukaemia Treatment.
Chronic Lymphocytic Leukaemia Treatment.
Adult Acute Myeloid Leukaemia Treatment.
Childhood Acute Myeloid Leukaemia/Other Myeloid Malignancies Treatment.
Chronic Myelogenous Leukaemia Treatment.

Gender and age may affect the risk of developing hairy cell leukaemia.

The cause of hairy cell leukaemia is unknown. It occurs more often in older men.

Possible signs of hairy cell leukaemia include tiredness, infections, and pain below the ribs.

These and other symptoms may be caused by hairy cell leukaemia. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

Weakness or feeling tired.
Fever or frequent infections.
Easy bruising or bleeding.
Shortness of breath.
Weight loss for no known reason.
Pain or a feeling of fullness below the ribs.
Painless lumps in the neck, underarm, stomach, or groin.

Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukaemia.

The following tests and procedures may be used:

Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as a swollen spleen, lumps, or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

Peripheral blood smear: A procedure in which a sample of blood is checked for cells that look "hairy," the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells.
Bone marrow biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for signs of cancer.
Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to check the pattern of proteins that are on the surface of the cells. Hairy cells have a certain pattern.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerised tomography, or computerised axial tomography. A CT scan of the abdomen may be done to check for swollen lymph nodes or a swollen spleen.

Certain factors affect treatment options and prognosis (chance of recovery).

The treatment options may depend on the following:

The number of hairy (leukaemia) cells and healthy blood cells in the blood and bone marrow.
Whether the spleen is swollen.
Whether there are symptoms of leukaemia, such as infection.
Whether the leukaemia has recurred (come back) after previous treatment.

The prognosis (chance of recovery) depends on the following:

Whether the hairy cell leukaemia does not grow or grows so slowly it does not need treatment.
Whether the hairy cell leukaemia responds to treatment.

Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukaemia are gone). If the leukaemia returns after it has been in remission, retreatment often causes another remission.

Stages of Hairy Cell Leukaemia

Staging is the process used to find out how far the cancer has spread. There is no standard staging system for hairy cell leukaemia. The disease is grouped as untreated, progressive, or refractory.

Untreated hairy cell leukaemia

The hairy cell leukaemia is newly diagnosed and has not been treated except to relieve symptoms such as weight loss and infections. In untreated hairy cell leukaemia, some or all of the following conditions occur:

Hairy (leukaemia) cells are found in the blood and bone marrow.
The number of red blood cells, white blood cells, or platelets may be lower than normal.
The spleen may be larger than normal.

Progressive hairy cell leukaemia

In progressive hairy cell leukaemia, the leukaemia has been treated with either chemotherapy or splenectomy (removal of the spleen) and one or both of the following conditions occur:

There is an increase in the number of hairy cells in the blood or bone marrow.
The number of red blood cells, white blood cells, or platelets in the blood is lower than normal.

Relapsed or Refractory Hairy Cell Leukaemia

Relapsed hairy cell leukaemia has come back after treatment. Refractory hairy cell leukaemia has not responded to treatment.

Treatment Option Overview

Key Points for This Section

There are different types of treatment for patients with hairy cell leukaemia.
Four types of standard treatment are used:

Other types of treatment are being tested in clinical trials. These include the following:

There are different types of treatment for patients with hairy cell leukaemia.

Different types of treatment are available for patients with hairy cell leukaemia. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Four types of standard treatment are used:

Watchful waiting

Watchful waiting is closely monitoring a patient's condition, without giving any treatment until symptoms appear or change.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukaemia.

Biologic therapy

Biologic therapy is a cancer treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent commonly used to treat hairy cell leukaemia. For relapsed or refractory patients, a biologic agent called rituximab may be used.

Surgery

Splenectomy is a surgical procedure to remove the spleen.

Other types of treatment are being tested in clinical trials. These include the following:

Stem cell transplant

Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer or cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a brother or sister and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Treatment Options for Hairy Cell Leukaemia

Untreated Hairy Cell Leukaemia

If the patient's blood cell counts are not too low and there are no symptoms, treatment may not be needed and the patient is carefully watched for changes in his or her condition. If blood cell counts become too low or symptoms appear, initial treatment may include the following: