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Hairy Cell Leukaemia: Symptoms, Diagnosis and Treatment of a Rare Blood Cancer
By Dr. Gajendra Kumar Himanshu
Hairy cell leukaemia is a rare type of blood cancer that affects abnormal B-lymphocytes, a type of white blood cell. These abnormal cells build up in the blood, bone marrow, and spleen, leaving less space for healthy blood cells. It usually develops slowly and may cause tiredness, frequent infections, easy bleeding, or pain and fullness below the ribs.
In this blog, we will explain hairy cell leukaemia in simple words, including its symptoms, possible risk factors, diagnosis, treatment options, and follow-up care. We will also explain why some people may not need treatment immediately and how doctors decide when treatment is needed.
What Is Hairy Cell Leukaemia?
Hairy cell leukaemia is a rare cancer of the blood and bone marrow. It affects B-lymphocytes, which normally help the body fight infections. Under a microscope, the abnormal cells have tiny hair-like projections, which is where the disease gets its name.
The abnormal cells can collect in the bone marrow and interfere with the production of healthy red blood cells, white blood cells, and platelets. They may also collect in the spleen, causing it to become enlarged.
Because it is a type of blood cancer, understanding how blood cancers affect the body can be helpful. You can read this simple blog on blood cancer symptoms.
How Does Hairy Cell Leukaemia Affect the Body?
The bone marrow is the soft tissue inside some bones where blood cells are made. In hairy cell leukaemia, abnormal B-lymphocytes build up in the bone marrow.
This can reduce the production of healthy blood cells. A low red blood cell count can cause anaemia and tiredness. A low number of healthy white blood cells can increase the risk of infections, while a low platelet count can make bruising or bleeding more likely.
The spleen can also become enlarged because it collects abnormal blood cells. This may cause pain or a feeling of fullness on the upper left side of the abdomen.
Symptoms of Hairy Cell Leukaemia
Hairy cell leukaemia can develop slowly, and some people may have few or no symptoms at first. Symptoms can also be similar to those caused by other health problems.
Common symptoms may include:
- Feeling weak or very tired
- Frequent infections
- Easy bruising or bleeding
- Shortness of breath
- Pain or fullness below the ribs
- Unexplained weight loss in some patients
- Swollen lymph nodes in some cases
An enlarged spleen may make a person feel full after eating only a small amount of food. If symptoms continue or keep returning, a doctor may recommend blood tests to find the cause.
What Causes Hairy Cell Leukaemia?
The exact cause of hairy cell leukaemia is not clearly known. It develops when changes occur in the genetic material of certain blood cells, causing them to grow abnormally.
A BRAF gene change, especially the BRAF V600E variant, is commonly found in classic hairy cell leukaemia and can help doctors confirm the diagnosis and guide treatment.
Having a genetic change does not mean that the disease was necessarily inherited from a parent. Most patients do not have a clear family history of hairy cell leukaemia.
How Is Hairy Cell Leukaemia Diagnosed?
Doctors usually begin with a physical examination and blood tests. A complete blood count (CBC) can show whether red blood cells, white blood cells, or platelets are lower than normal. Doctors may then use more specific tests to identify the abnormal cells.
Diagnostic test | What it helps doctors find |
|---|---|
Complete blood count | Checks red blood cells, white blood cells, and platelets |
Blood smear | Looks at the shape and appearance of blood cells |
Flow cytometry | Identifies specific markers on abnormal cells |
Bone marrow biopsy | Checks the bone marrow for hairy cells |
BRAF testing | Looks for common genetic changes linked with the disease |
CT scan | Can check for an enlarged spleen or lymph nodes |
Flow cytometry is particularly useful for identifying the abnormal B cells. A bone marrow biopsy may also be needed, especially when doctors need more information about the disease.
Is There a Stage System for Hairy Cell Leukaemia?
Unlike many solid cancers, hairy cell leukaemia does not have a standard staging system. Doctors instead look at factors such as blood cell counts, the number of abnormal cells, spleen size, symptoms, and whether the disease has responded to earlier treatment.
The disease may also be described as recurrent when it returns after treatment or refractory when it does not respond adequately to treatment.
Treatment for Hairy Cell Leukaemia
Treatment is not always needed immediately. If a person has no significant symptoms and blood counts remain at an acceptable level, doctors may choose watchful waiting. This means regular check-ups and blood tests while treatment is held until there are signs that treatment is needed.
When treatment is needed, the choice depends on the patient’s symptoms, blood counts, spleen size, previous treatment, overall health, and other factors.
Chemotherapy
Medicines such as cladribine and pentostatin have been used to treat hairy cell leukaemia. Cladribine, with or without rituximab, remains an important treatment option for many patients. However, treatment can lower blood counts and increase the risk of infection, so doctors carefully consider the patient’s condition before choosing therapy.
If chemotherapy is recommended, understanding possible side effects can help patients prepare. You can read this guide on common chemotherapy side effects and their management.
Targeted Therapy
Targeted therapy works on specific features that help cancer cells survive or grow. BRAF inhibitors may be considered for patients whose cancer has the relevant BRAF change. Medicines such as vemurafenib or dabrafenib may be used in selected cases, sometimes along with other treatments.
Immunotherapy and Antibody Treatment
Rituximab is a monoclonal antibody that targets CD20, a protein found on the surface of many B cells. It may be used alone or together with other treatments in selected patients.
Surgery
Surgery to remove the spleen, called splenectomy, was used more often in the past. Today, it is used less frequently because effective drug treatments are available. It may still be considered in specific situations when other treatments are not suitable or do not work well.
Living With Hairy Cell Leukaemia
Many patients can have long periods of disease control after treatment. Because the disease can return, regular follow-up is important even when a person feels well.
Doctors may monitor blood counts and other tests to check whether the disease remains under control. Patients should also report repeated infections, unusual bleeding, worsening tiredness, or increasing abdominal fullness.
Treatment can sometimes weaken the immune system, so infection prevention and timely medical advice are important during and after treatment.
When Should You See a Doctor?
Symptoms such as tiredness, repeated infections, bruising, or abdominal discomfort can have many causes. They do not always mean leukaemia.
However, symptoms that persist or keep returning should be discussed with a doctor. Blood tests can often provide useful information and help doctors decide whether further testing is needed.
Early medical assessment is especially important when symptoms are accompanied by frequent infections, unusual bleeding, or significant weakness.
If you want to read more about hairy cell leukaemia, you can visit the National Cancer Institute.
Consult Today
Hairy cell leukaemia is rare, but it is often a slowly growing disease that can be managed with appropriate treatment and regular follow-up. The right treatment depends on the patient’s blood counts, symptoms, spleen size, genetic findings, overall health, and previous treatment.
At Oncare Cancer Hospital, patients with blood cancers can receive specialist care from a multidisciplinary team. Treatment may include chemotherapy, targeted therapy, immunotherapy, and other appropriate approaches depending on the diagnosis and individual needs.
Disclaimer
This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always discuss decisions about blood tests, medicines, or any cancer treatment with a certified cancer healthcare provider.
Frequently Asked Questions
Yes, hairy cell leukaemia is a rare type of blood and bone marrow cancer.
Common symptoms include tiredness, frequent infections, easy bruising or bleeding, shortness of breath, and pain or fullness below the ribs.
Yes, treatment can include watchful waiting, chemotherapy, targeted therapy, antibody treatment, and, in selected cases, surgery.
No, some people with no significant symptoms may be monitored without immediate treatment. Doctors start treatment when there are signs that the disease needs to be controlled.
Written and Verified by:
Dr. Gajendra Kumar Himanshu Exp: 10 Yr
Medical Officer
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