Hairy Cell Leukemia can be difficult to diagnose. Blood tests show that the bone marrow produces abnormal lymphocytes (white blood cells that help fight infection). These over-accumulate in the blood and crowd out healthy red blood cells and platelets, leading to anemia and easy bleeding.
Treatment usually puts the disease into remission. People in remission can live as long as people without the condition.
Hairy Cell Leukemia is caused by a change (or mutation) in genes that affects the bone marrow. This is the center inside the bones where blood stem cells develop into healthy red blood cells, platelets and white blood cells. With hairy cell leukemia, immature blood stem cells grow out of control and don’t mature into normal white blood cells. As a result, the blood can’t fight infections as well.
Infections and fatigue are common symptoms of hairy cell leukemia. People with this disease may also have a painless lump in the neck, underarm or stomach. In some cases, hairy cells leave the bone marrow and travel through the bloodstream to other organs. They often collect in the spleen, which is located on the upper left side of the belly and helps filter and remove worn-out blood cells from the body. When the spleen gets larger, it can press against other organs in the abdomen causing pain or a feeling of fullness.
The abnormal hairy cells also can collect in other parts of the body, including the lungs and liver. This can cause a feeling of being short of breath, problems with liver function and fluid buildup in the abdomen called ascites. Rarely, hairy cell leukemia can spread to the brain causing headaches and changes in thinking.
A complete blood count (CBC) test and a bone marrow biopsy are the main tests used to diagnose hairy cell leukemia. These tests check your levels of red blood cells, white blood cells and platelets. They also look for a high percentage of cells that are hairy under a microscope. A bone marrow biopsy takes a sample of the marrow, which is then examined under a microscope to check for hairy cells.
Chemotherapy is typically the first treatment for hairy cell leukemia. Cladribine (Leustatin(r)) and pentostatin (Nipent(r)) are the most commonly used anticancer drugs for this condition. These are given as short intravenous infusions every 2-3 weeks until a remission is achieved. In some cases, these drugs are followed by a monoclonal antibody therapy such as rituximab (Rituxan(r)). If a relapse occurs, your doctor may consider moxetumomab pasudotox (Lumoxiti(r)), a newer drug in combination with chemotherapy.
Hairy Cell Leukemia is a type of blood cancer that affects the white blood cells. This type of cancer occurs when cells develop mutations and begin growing out of control. It’s a rare form of leukemia that affects men more often than women. Doctors are able to diagnose this condition after learning about your medical history and doing a physical exam. The doctor may feel your spleen, liver and lymph nodes for signs of swelling.
During the physical exam, your provider will ask about symptoms you have and how long you’ve had them. The doctor will also do a complete blood count (CBC) test to check your red and white blood cells, platelets and other parts of your blood. The CBC can show reduced numbers of these cell types, which can cause fatigue and other problems. The CBC will also look for abnormal cells called leukemia cells. The leukemia cells are named for their tiny projections that give them a hairy appearance under a microscope.
Another type of test, a flow cytometry analysis, can identify the abnormal hairy cells in samples from your blood and bone marrow. This test can help your doctor decide what treatment is right for you.
In addition to these tests, your doctor may recommend a computed tomography scan, an ultrasound or other diagnostic procedures. During the CT scan, your doctor will take pictures of areas inside your body using an X-ray machine. The pictures will provide a clearer picture of your blood vessels, organs and bones. They may also order a CT scan of your abdomen to see if your spleen and liver are enlarged.
Because hairy cell leukemia grows slowly, doctors usually wait until you have symptoms before treating you with chemotherapy. This is known as active surveillance. Your hematologist or oncologist will examine you regularly and take blood tests to monitor your blood cell counts. You’ll need to have these tests done quarterly or more often if you have signs and symptoms. You can also choose to participate in a clinical trial that’s testing new treatments for hairy cell leukemia.
Patients with hairy cell leukemia have a disturbed immune system that causes symptoms such as infections (usually fever, fatigue, rash and joint pain), abnormal skin changes, bleeding in the digestive tract or nose, liver problems, swelling of the lymph nodes in the neck or abdomen and headaches. The cancerous cells can also invade bones, causing pain in the bone and in the joints and a build-up of fluid in the abdomen called ascites. Hairy cell leukemia has a very slow onset and may not be diagnosed until several months or even years after the first signs of illness.
In some people, the condition is very mild and does not cause any symptoms. These people are often referred to as “watch and wait.” They are monitored closely with blood tests and a physical exam to check for low levels of red and white blood cells, and platelets (cytopenias). If the leukemia is still present and the symptoms persist, treatment may be needed.
The most common treatments are chemotherapy drugs, such as cladribine or pentostatin. These medications enter the bloodstream and reach all parts of the body to kill cancer cells. They can be taken by mouth or injected into a vein or muscle (intravenous).
Both of these medications have been shown to improve blood counts and produce remission in hairy cell leukemia. They are very effective front-line therapy. However, these drugs can increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma, so long-term follow up to look for second cancers is important.
Other options for treating hairy cell leukemia include a steroid medication called prednisone and immunosuppressive agents. In addition, new types of treatment are being tested in clinical trials. These are studies that evaluate whether a new treatment is safe and helps the disease get better. For information about ongoing clinical trials, visit the NCI website.
Because hairy cell leukemia is rare, it can be difficult for doctors to know how often it comes back (relapses) and how long people with it may live. However, research shows that treatment can put the disease into remission for 10 years or more. Remission means that you don’t have any symptoms and tests show no signs of the disease.
Your doctor will ask you about your past health and do a physical exam. They will feel your lymph nodes, spleen, and liver for signs of swelling. Then they will order a blood test to check your white blood cells and platelets. This is called a complete blood count, or CBC. It will also show whether your red blood cells are healthy or have too few of them. A CBC with differential can identify the type of white blood cell that is affected in hairy cell leukemia, and will look for the small, hair-like projections that give this condition its name. A peripheral blood smear may also be done to look for these abnormal cells.
These tests can help your doctor decide the best way to treat you. For example, your doctor might use chemotherapy alone or with other treatments, such as radiation therapy or a monoclonal antibody. They might also use a combination of drugs called targeted therapy. Targeted therapy uses drugs that go after certain kinds of cancer cells and stop them from growing.
If you have hairy cell leukemia, you might also need a scan to see how your spleen and lymph nodes are doing. A CT scan is an imaging test that makes a series of detailed pictures of areas inside your body, including your organs and tissues. A special dye helps your spleen and lymph nodes show up better on the images. Your doctor might also recommend a bone marrow biopsy to find out how well the chemotherapy is working.
Because hairy cell leukemia isn’t curable, your doctor will usually wait until you have symptoms before starting treatment. This is called active monitoring and can help you avoid the side effects of treatment until it’s needed.
A change (mutation) in genes causes the blood stem cells to produce too many white cells that don’t work right. These hairy cell leukemia cells collect in the bone marrow, liver and spleen, where they can enlarge and crowd out healthy white cells, red cells and platelets.
Doctors can diagnose this type of leukemia by asking about your symptoms, doing a physical exam and ordering certain tests. These may include a peripheral blood smear, bone marrow aspiration and/or a CT scan (CAT scan).
Hairy Cell Leukemia affects B cells (also known as B lymphocytes). These are the types of white blood cells that fight infections. When these abnormal cells take over your bone marrow, they crowd out healthy white blood cells and other blood stem cells. This can lead to low levels of all three kinds of blood cells — red blood cells, platelets and white blood cells. This can cause you to feel tired and have a loss of appetite. It can also cause your spleen to swell. It may be painful in your lower left side or it can press against your abdomen (belly). This is a common sign of this type of leukemia.
Your healthcare providers will check you for these symptoms. They’ll ask you about your past health problems and do a physical exam. They’ll feel (palpate) your lymph nodes, spleen and liver for signs of swelling.
They’ll also do some blood tests. They’ll include a complete blood count and a peripheral blood smear. These tests can help your doctors find out if you have low blood counts, and they can look for the abnormal ‘hairy’ cells that give this condition its name. A specialized test, called flow cytometry, can also help identify these cells. It looks for changes in the shape and color of these cells, as well as their ability to make certain proteins.
Your doctor might also use a CT scan or an ultrasound to check your spleen and liver. They may also do a biopsy to remove cells from your spleen or liver. They can then examine them under a microscope to see if they have the tiny hair-like projections that characterize this leukemia.
The malignant hairy cells in the bone marrow interfere with the usual production of red blood cells (that carry hemoglobin), white blood cells, and platelets. This decrease in blood cell production causes extreme tiredness. It also reduces the number of white blood cells, which weakens your immune system. This makes you more susceptible to infections and increases your risk of catching pneumonia or bronchitis.
In addition, the spleen, an organ in your body that filters blood from your digestive tract, may enlarge and cause pain or a feeling of fullness in the stomach. This problem can be a very early sign of hairy cell leukemia, and it’s usually treated with medications.
A simple blood test called a complete blood count can help doctors diagnose Hairy Cell Leukemia. It shows a lower-than-normal level of red blood cells, a low white blood cell count that increases your risk of infection, and a reduced number of platelets that may lead to abnormal bleeding. This blood work can be done in a laboratory at NYU Langone.
If your doctor suspects you have hairy cell leukemia, they’ll perform a physical exam and ask about your past health history. They may recommend a CT scan or ultrasound of your spleen and liver to look for signs of enlargement. They will also order a blood test that checks for a higher-than-normal number of hairy cells and a decreased amount of another type of white blood cell, known as monocytes. If the tests indicate you have hairy cell leukemia, our hematologists will give you chemotherapy with either cladribine or pentostatin, which can put your cancer into remission. In remission, your symptoms will disappear.
Bloody diarrhea can be a symptom of many health conditions. It may be caused by an infection like colitis, a condition in the intestines, or it can be a side effect of medication. In some cases, it can be a sign of cancer.
In hairy cell leukemia, a type of blood cancer, cells in the bone marrow produce abnormal ones that do not develop properly. These cells then travel through the bloodstream to other parts of the body, such as the liver, spleen and lymph nodes. Bloody diarrhea can be a symptom that these cells have gathered in the spleen.
The cells can also make your spleen two to three times larger than normal, causing pain on the left side of your abdomen (belly) or an early feeling of fullness in your stomach. Hairy cell leukemia can also keep your bone marrow from making enough platelets, which are the blood cells that help to stop bleeding. This can lead to nosebleeds, gum or mouth bleeding, or severe bleeding from the stomach.
Hairy cell leukemia typically gets worse slowly, so treatment might not be needed right away. However, NYU Langone hematologists-doctors who study and treat diseases of the blood-can usually manage to get this form of leukemia into remission, where the signs and symptoms of the condition decrease or disappear. If your doctor thinks you have this condition, they might order other tests to check your levels of blood cells. These might include a complete blood count, an imaging test such as a computed tomography (CT) scan, or other tests to look for other causes of the symptoms. Your doctor might also refer you to a specialist in the treatment of blood and bone marrow disorders.
Hairy Cell Leukemia (HCL) starts in the bone marrow, which is the soft tissue inside your bones where blood cells are made. It happens when a change in your genes causes your bone marrow to make too many white blood cells called B lymphocytes. These abnormal B cells don’t work right and crowd out healthy blood cells that fight infections. As a result, you may have more infections and easier bruising and feel tired. This type of cancer is rare and typically affects older adults. It is more common in men than women.
There are different types of treatments for hairy cell leukemia. These include chemotherapy and other drugs to shrink cancerous cells. Most people with this cancer get a remission, which means the cancer disappears for several years. However, the cancer can come back after treatment. When this happens, you have refractory cancer or resistant cancer. A new drug called moxetumomab pasudotox has been shown to help treat this cancer.
If you have hairy cell leukemia, your doctor will want to check your blood and spleen. They will also look at your medical history and do a physical exam. They will ask questions about your symptoms and when they started.
The first test to diagnose hairy cell leukemia is a complete blood count. This test measures the levels of red blood cells, white blood cells, and platelets in your blood. It will also show whether you have a low platelet count, which can lead to bleeding problems. A peripheral blood smear is another important test for this condition. It is a sample of blood from a finger prick, and doctors can examine it under a microscope to look for the abnormal “hairy” cells that cause this disease.
Patients with Hairy Cell Leukemia develop infections that are caused by problems with the blood cells. These infections may affect the skin, lungs or liver and may cause symptoms such as fever, cough or rashes. They may also cause bleeding in the spleen, which can cause pain on the left side of the abdomen or a feeling of early fullness after eating. Infections can occur in other organs as well, including the brain.
Hairy Cell Leukemia occurs when the bone marrow produces too many immature white blood cells called B lymphocytes. These lymphocytes don’t mature into healthy blood cells and crowd out other types of cells that produce red blood cells, platelets and white blood cells. This can result in low levels of all three blood cells (anemia), a condition that can make it harder to fight off infections, and/or low numbers of healthy platelets, which can lead to bleeding problems.
In addition to the problems with the blood cells, Hairy Cell Leukemia can interfere with the production of normal hormones, such as insulin and growth factors. These abnormalities can cause a loss of weight and muscle tissue, which some people find disturbing.
Symptoms of Hairy Cell Leukemia are usually gradual in onset and often go unnoticed until the disease becomes more serious. This can lead to delays in diagnosis. In some cases, doctors may choose not to treat patients with Hairy Cell Leukemia if their blood counts deteriorate but do not reach very low, dangerous levels. This approach is called active monitoring and helps avoid the side effects of treatment if it is not needed. The decision to re-start treatment is made after careful consideration if the symptoms return.
Most people with hairy cell leukemia get chemotherapy drugs to treat their cancer. These medications may help relieve symptoms and improve blood counts.
Your doctor will ask about your past health and symptoms and do a physical examination. Your doctor will also order blood tests and a bone marrow biopsy.
Hairy cell leukemia is rare, and it can be hard to diagnose. Doctors may suspect this type of cancer if you have symptoms such as fatigue, weight loss, pain in the lower left side of the abdomen (splenomegaly), swollen lymph nodes, and unusual bruising or bleeding. Doctors can find the cause of these symptoms by doing a physical exam, asking about your health history, and ordering blood tests. These can include a complete blood count with differential, which measures the levels of red blood cells, white blood cells, and platelets in your body. If you have hairy cell leukemia, the CBC test will show that all of these levels are too low.
Your doctor may also order an X-ray or ultrasound to check for enlarged organs, such as the spleen and liver. If these organs become too large, they can press against the intestines or stomach, causing pain, discomfort, and a feeling of fullness. They can also block the flow of blood to these organs, causing complications.
Blood tests can help doctors diagnose hairy cell leukemia by detecting the abnormal B lymphocytes that are characteristic of this condition. These cells have distinctive projections that give them a “hairy” appearance. The test can also find other problems that occur with hairy cell leukemia, such as a low level of healthy red blood cells or platelets.
If you have hairy cell leukemia, your doctors may use a bone marrow biopsy to confirm the diagnosis and make sure the cancer has not spread beyond the marrow. During this procedure, doctors inject a numbing medication into the back of your pelvis and remove a sample of bone marrow. They then look at the marrow under a microscope to see if there are any hairy cells.
Some people with hairy cell leukemia have no symptoms at all, and the disease is only found by chance when a blood test is done for another reason. In this case, your doctor may recommend watchful waiting and monitor you carefully without treatment. This is sometimes called active surveillance.
When hairy cell leukemia is present in the bone marrow, it causes an overproduction of abnormal blood cells. These cells crowd out the healthy blood stem cells and other normal blood cells that normally produce red blood cells (erythropoiesis), white blood cells that fight infection, and platelets that stop or slow bleeding. This results in low levels of these blood cells, causing symptoms and complications such as anemia, swollen lymph nodes (lymphadenopathy), fatigue, and easy bruising and bleeding.
Hairy cell leukemia typically starts in the bone marrow but can spread to other organs including the liver and spleen. A common symptom of the disease is an enlarged spleen, usually on the left side, that can be two to three times larger than normal and cause pain on the left side of the abdomen, a feeling of fullness in the stomach early, and bruising and/or severe bleeding.
A splenectomy is often performed to remove the enlarged spleen. This can improve the blood counts in some patients but is not a cure and the spleen may re-enlarge over time. The underlying bone marrow problems that lead to the hairy cell leukemia usually remain unchanged.
Currently, the most effective treatment for hairy cell leukemia is the drug cladribine. It is a chemotherapeutic drug that can be given in pill form or intravenously. The drug works by blocking the production of new blood cells by preventing them from growing. It is generally well tolerated by most patients. A remission is seen in about 90 percent of the patients who receive cladribine.
For those patients that do not achieve a remission after receiving cladribine, a different treatment option should be considered. The BRAF inhibitors vemurafenib and dabrafenib combined with the monoclonal antibody rituximab (often administered as an IV infusion) are very effective in most cases, especially when the BRAF mutation status is known and verified. This combination has also been successful in relapsed hairy cell leukemia patients who have failed to respond to prior therapies. If re-treatment is started, care should be taken not to let the blood counts fall too low to dangerous levels.
The slow onset of symptoms and rarity of hairy cell leukemia make accurate diagnosis difficult. Physicians should be very careful to use their clinical judgement when evaluating patients with this disease. They should also note that in addition to bone marrow involvement, the disease commonly travels to other organs. For example, a patient with hairy cell leukemia may develop an enlarged spleen that is two to three times the normal size. This can cause pain on the left side of the abdomen where the spleen is located, early feeling of fullness when eating and in severe cases can lead to spontaneous bleeding, particularly from the stomach.
To diagnose the condition, healthcare providers will perform a physical exam and listen to the patient’s story. They will also order blood tests to look at the level of different types of cells in the blood, including red blood cells, white blood cells and platelets. They may also order a bone marrow aspiration and a biopsy to examine the inside of the bones for signs of abnormal cells.
These tests can help determine whether or not the patient needs treatment right away. In most cases, however, it is appropriate to begin treatment if the blood counts indicate that the leukemia is active. This can be very distressing for the patient, especially their loved ones. The medical team can ease some of the anxiety by reassure patients that they can continue to be monitored closely and that they will only be given treatment when the symptoms suggest it is needed.
Typical front-line treatment for hairy cell leukemia is a seven-day course of intravenous infusions of the purine nucleoside analog cladribine with or without eight weekly doses of rituximab. These agents achieve high levels of remission in most patients with hairy cell leukemia and are associated with minimal toxicities. Unfortunately, for some patients the disease will relapse or will be unresponsive to cladribine or pentostatin. These patients will typically be retreated with another form of the same drug or another option such as fludarabine in combination with rituximab or oral BRAF inhibitor vemurafenib.
Hairy cell leukemia is a type of cancer that affects B cells, which are types of white blood cells that make antibodies to fight infections. It gets its name from the unique shape of these cells under a microscope, which look like tiny hairs. This cancer is slow growing, and it often doesn’t cause symptoms or need treatment right away.
In fact, many people with this cancer live for years without needing treatment. But if the cancer does start to grow, your healthcare provider may want to start treatment right away. This is because some of the treatments for this cancer are very effective, and most people with hairy cell leukemia go into remission after they receive them.
Your healthcare provider will check your overall health to see if you have any other serious health conditions that could impact how well treatment works. They will also perform a blood test to check your white blood count. If this test comes back high, your healthcare provider may want to do a bone marrow biopsy to get a better sample of your leukemia cells.
Using a special type of microscope, pathologists will examine your peripheral blood and bone marrow for abnormal hairy cells. They will also do other tests to check the size of your spleen and lymph nodes, and they will look for tumor markers, which are proteins that certain cancerous cells make.
If your health care provider thinks you have hairy cell leukemia, they might recommend you see a specialist, such as a hematologist or oncologist. They can help you decide if you need treatment and what treatment might be best for you.
If you’ve already been treated for hairy cell leukemia, your doctor will continue to monitor your blood counts regularly. They will look for signs that the leukemia is relapsing, such as lower than normal white blood cell levels or low platelet counts. They may also do a flow cytometric analysis of your blood, bone marrow and/or spleen samples to identify recurrent hairy cells. These tests will check for specific characteristics of the leukemia cells, including morphology (the shape of the cells) and immunophenotyping (looking at the kinds of markers on the surface of the cells). They will also look for the BRAFV600E mutation in these cells.
