Hairy Cell Leukaemia: Symptoms, Causes, Images, Treatment

Heard about Hairy Cell Leukaemia (HCL)? Yes, there is a cancer known as Hairy cell leukaemia. It is a rare form cancer of the blood and bone marrow that develops when the bone marrow develops abnormal white blood cells. 

Advancements in the treatment of hairy cell leukaemia have brought new hope and improved outcomes allowing for long-term management of the disease for individuals diagnosed with this rare blood cancer.  

The following article discusses the hairy cell leukaemia pathology outlines, symptoms, causes, diagnosis, and treatment for a better understanding of the disease. Continue reading to know more. 

Hairy cell leukaemia is a rare type of chronic lymphocytic leukemia (CLL), which involves the overproduction of a specific type of white blood cell (WBC) called B lymphocytes or B cells. The name “hairy cell” comes from the appearance of the cancerous cells under a microscope. These cells have fine, hair-like projections on their surface, resembling hair. 

HCL is chronic cancer, i.e., it may progress very slowly, and people may have this condition for many years without developing any symptoms. As a result, hairy cell leukaemia treatment may not be immediately needed.

While HCL is generally classified as a single disease, there are different subtypes or variants that have been identified. Here are the different types of hairy cell leukemia:

1. Classic Hairy Cell Leukaemia: This is the most common subtype of HCL, accounting for the majority of cases. It is characterized by abnormal B cells with hair-like projections on their surface. Classic HCL is typically associated with a good prognosis and responds well to treatment.

2. Variant Hairy Cell Leukaemia: Also known as HCL variant or HCLv, this subtype is less common than classic HCL. It shares some similarities with classic HCL but also exhibits distinct features. Variant HCL cells have fewer hair-like projections, and the disease is generally more aggressive and less responsive to treatment compared to classic HCL.

The symptoms of hairy cell leukaemia can vary from person to person, and some individuals may not experience any symptoms at the early stages of the disease. However, as the condition progresses, the following symptoms may commonly occur:

1. Fatigue or Weakness: Hairy cell leukaemia can cause a persistent feeling of tiredness and weakness, which may interfere with daily activities.

2. Frequent Infections: HCL affects the immune system, making individuals more susceptible to infections.

3. Easy Bruising or Bleeding: HCL can result in easy bruising or bleeding from minor injuries, such as frequent nosebleeds, bleeding gums, or prolonged bleeding after cuts.

4. Fever or Night Sweats: Some individuals with HCL may experience recurrent fevers and night sweats.

5. Loss of Appetite: Due to the decreased production of normal blood cells, individuals with HCL may experience fatigue and a reduced desire to eat. 

6. Unintentional Weight Loss: HCL patients may experience unintentional weight loss due to a decreased appetite and the body’s response to the disease. 

7. Shortness of Breath: It can occur due to anaemia, where there is a decrease in red blood cells and oxygen-carrying capacity. 

8. Pain or Fullness in the Belly just under the Ribs: Individuals may develop discomfort or a feeling of fullness in the belly due to an enlarged spleen. 

9. Painless Lumps in the Underarm, Neck, Stomach, or Groin: These can occur due to enlarged lymph nodes or an enlarged spleen.

According to some research, hairy cell leukaemia most commonly affects men more than women and middle-aged and older people. The exact cause of this condition is not yet fully understood. However, it develops when there are changes in the DNA of an individual’s B cells. The DNA contains instructions, informing the cell what to do. 

1. Healthy cells die as part of the natural cell life cycle. However, changes in the DNA cause the B cells to continue growing.  

2. Abnormal B cells crowd out healthy blood cells in the bone marrow, causing symptoms and complications associated with the disease.

Hairy Cell Leukaemia Risk Factors

While the cause of HCL is unclear, certain factors can raise the risk of developing the disease. However, having these risk factors does not necessarily mean a person will develop HCL. These risk factors include:

1. Age: Hairy cell leukaemia can develop at any age. However, it mostly affects people between the ages of 40 and 70. The condition is rare in children. 

2. Gender: HCL is more common in men than in women. Men are approximately five times more likely to develop HCL than women.

3. Ethnicity: The condition is more common in people who are white than other ethnic groups. 

4. Family History: An individual’s risk of developing HCL is slightly higher if they have a close relative (parent or sibling) with this disease. However, most people with a family history of the disease do not develop the condition themselves.

5. Exposure to Chemicals: An individual is at a higher risk of developing HCL if he/she is exposed to chemicals like benzene.

At present, there are no known specific measures for preventing the development of hairy cell leukaemia. Since the exact causes of HCL are not fully understood, there are no targeted strategies for its prevention. However, the following preventive measures can help reduce the risk of the disease: 

1. Avoid Smoking and Drinking Alcohol: Cigarette smoking and alcohol consumption have been linked to an increased risk of various cancers, including leukaemia. Quitting smoking and limiting alcohol intake is crucial for reducing the risk of developing HCL.

2. Avoid Exposure to Harmful Chemicals: Minimising exposure to potentially harmful chemicals, like pesticides and solvents, can benefit overall health. Individuals who work in places with chemical exposure should follow safety guidelines and use protective measures. 

3. Regular Medical Checkups: Medical checkups at frequent intervals allow for early diagnosis and management of potential health concerns.

4. Maintain a Healthy Lifestyle: Regular exercise, a balanced diet, adequate sleep, and stress management may help support the immune system, which is vital for combating diseases.

Patients may need to visit a haemotologist (a doctor specialising in blood disorders) to get the right diagnosis. Doctors understand the hairy cell leukaemia pathology by performing the following test and procedures to diagnose the condition:

1. Physical Exam: Healthcare professionals will perform a physical examination to look for signs of HCL, such as an enlarged spleen or lymph nodes. They may be able to feel an enlarged spleen during the test. 

2. Complete Blood Count (CBC) with Differential: The test measures the levels of platelets and blood cells (red blood cells and white blood cells). In HCL, CBC shows low levels of all blood cells.

3. Peripheral Blood Smear: In this test, the doctors look at the patient’s blood cells under a microscope. Hairy cell leukaemia pathology outlines include cells that have a hairy appearance. 

4. Bone Marrow Tests: Doctors perform these tests to look for signs and symptoms of hairy cell leukaemia and to measure the number of blood cells in the bone marrow. Bone marrow testing involves two steps: a bone marrow aspiration (to remove a sample of liquid marrow) and a bone marrow biopsy (to remove a sample of bone marrow tissue). 

5. Gene Mutation Test: The test examines a blood or tissue sample for changes in the BRAF gene (offers instructions for making a protein that transmits chemical signals from outside a cell to the cell’s nucleus). A BRAF gene mutation is usually found in HCL patients. 

6. Immunophenotyping: It is a laboratory test that uses antibodies to identify cancerous cells based on hairy cell leukaemia markers or antigens on the surface of the cells. The unusual formation of these antigens can indicate leukaemias. 

7. Flow Cytometry: Healthcare professionals perform this test to determine the number of cells and the percentage of living cells in a blood sample. The sample also measures cell characteristics, such as the size, shape, and presence of tumour markers on the cell surface. 

8. Cytogenetic Analysis: In this laboratory test, healthcare professionals test blood and bone marrow samples to count and check the chromosomes of cells. The test may reveal broken, rearranged, missing, or extra chromosomes, indicating cancer. It may also look for hairy cell leukaemia translocation (a portion of a chromosome breaks off and attaches to another chromosome), which is rare. 

9. Computed Tomography (CT) scan: A CT scan shows detailed pictures of the inside of the body. The test may help look for spleen, liver, and lymph node swelling. 

How to Prepare for Doctor’s Consultation?

Preparing for a doctor’s consultation for hairy cell leukaemia can ensure the patient makes the most of their appointment and gather all the necessary information. Here are some tips to help them prepare:

1. Write Down the Symptoms: Before the appointment, the patient must note any symptoms they have been experiencing, even if they seem unrelated. Include details like when the symptoms started, how often they occur, and if anything makes them better or worse. This information will help the doctor assess their condition more accurately.

2. Write Personal Information: The doctor may inquire about the patient’s medical history, including any previous illnesses or surgeries. They must include any major stresses or recent life changes. 

3. Make a List of Medications: The patients must list all their medications, including prescribed, over-the-counter, vitamins, supplements, and herbs. This helps the doctor understand if there are any drug interactions. 

4. Prepare a List of Questions: It is recommended to write down any questions or concerns the patient may have about HCL, its treatment options, potential side effects, and long-term outlook. It is easy to forget things during the appointment, so having a list ensures they cover all their concerns and receive the necessary information.

5. Bring a Trusted Companion: The patient may benefit from bringing a friend or family member to the appointment. They can provide support, help remember important details discussed during the consultation, and ask questions.

What to Expect from the Doctor?

During a consultation appointment for hairy cell leukaemia, the doctor may ask various questions to gather information about the patient’s symptoms, medical history, and overall health. Some potential questions the doctor may ask include:

1. What symptoms are you experiencing?

2. When did you begin having symptoms?

3. How severe are your symptoms?

4. Are the symptoms continuous or occasional?

5. Does anything seem to improve or worsen your symptoms?

6. Have you noticed any enlargement or tenderness in your spleen or lymph nodes?

7. Have you noticed any unusual bruising, bleeding, or recurrent infections?

What Questions to Ask the Doctor?

During a consultation appointment for hairy cell leukaemia, patients must actively engage with their doctor and ask questions to gather information and better understand their condition. Here are some questions they may consider asking during the appointment:

1. What kind of tests will I need for diagnosis?

2. Will I need treatment for hairy cell leukaemia?

3. If I don’t get treatment, will my condition worsen?

4. If I do get treatment, what are my treatment options? Which one do you recommend?

5. What is the goal of the treatment?

6. Will treatment cure my leukaemia?

7. What are the chances of success of treatment?

8. What are the risks and benefits associated with each option?

9. How will the treatment be administered, and what can I expect during the treatment process?

10. Will I need to follow any restrictions?

11. How will the HCL treatment affect my daily life?

12. How will it affect my other health conditions?

13. Should I visit a specialist?

Patients must feel free to ask any other questions that come to mind during the consultation.

Hairy cell leukaemia treatment typically aims to achieve long-term remission, manage symptoms, and improve the overall quality of life. The specific hairy cell leukaemia treatment guidelines depend on factors such as:

1. Number of healthy blood cells vs hairy leukaemia cells in the blood and bone marrow.

2. The presence of enlarged spleen.

3. Signs and symptoms of leukaemia, such as an infection, fever, night sweats, etc. 

4. Leukaemia recurrence after previous treatment.

If HCL grows slowly and does not cause symptoms, the doctor may recommend watchful waiting before starting a treatment plan immediately. It involves careful monitoring of the person’s condition and symptoms with regular physical exams and lab tests. Most people with HCL will eventually need treatment. Indications for treatment include:

1. Haematologic Parameters

1. Haemoglobin less than 11 g/dL

2. Platelet count less than 100 000 /μL

3. Absolute neutrophil (white blood cell that helps the body fight infection) count less than 1000 /μL

2. Clinical Features or Symptoms

1. Symptomatic organomegaly (enlarged liver or spleen)

2. Progressive lymphocytosis (an increase in the lymphocyte count)

3. Lymphadenopathy (swollen lymph nodes)

4. Unexplained weight loss (more than 10% within six months)

5. Excessive fatigue

The primary treatment options for HCL include:

1. Chemotherapy: It is usually the first-line treatment for hairy cell leukaemia. This effective treatment uses powerful drugs to kill cancer cells. Most people achieve partial or complete remission (no signs of cancer) after chemotherapy. The commonly used chemotherapy drug for HCL is cladribine, given intravenously. 

2. Targeted Therapy: It uses drugs to attack specific proteins and genes involved in cancer cell growth. It may sometimes be used as a first-line treatment for hairy cell leukaemia with chemotherapy or in its place. Targeted therapy may also be used if cancer comes back after chemotherapy. 

3. Immunotherapy: The treatment helps the person’s immune system fight cancer. Two common immunotherapies for HCL include interferon and rituximab. Immunotherapy for HCL is often used with chemotherapy, particularly in cases of relapsed/refractory disease. 

4. Splenectomy: In certain situations, if the spleen becomes significantly enlarged or if it causes severe symptoms, surgical spleen removal (splenectomy) may be recommended. It will not treat HCL but can help blood count to return to normal. Surgery for HCL is extremely uncommon. 

Please Note: The selection of treatment procedure is based on the patient’s health condition and the treating doctor’s opinion. 

Cost of Hairy Cell Leukaemia Treatment

The cost of HCL treatment in India is influenced by several factors including the type of HCL, HCL stage, treatment technique used, patient’s age, patient’s comorbidites, hospital type and location, doctor’s expertise, insurance coverage and much more.

To know the exact cost of the HCL treatment in India, one must consult their doctor. However, the following table also provides the approximate cost for various HCL treatment modalities.

Hairy Cell Leukaemia Survival Rate

The survival rate is a statistical indicator that shows the proportion of persons with a particular ailment who continue to live after being diagnosed for a specific amount of time.

Thanks to developments in treatment choices, hairy cell leukaemia (HCL) survival rates have risen significantly over time. About 90% of people who receive initial chemotherapy treatment with cladribine have a five-year event-free survival rate. This means that 90% of people with HCL will still be alive five years after diagnosis.

Treatment with cladribine has resulted in approximately 85% complete remission and around 10% partial response in HCL patients. The prognosis for HCL is generally favourable, and many individuals with HCL can achieve long-term remission and live a normal life span.

Hairy cell leukaemia often progresses very slowly. This is why only a few complications may occur. These complications can arise from abnormal growth and accumulation of hairy cells in the bone marrow and other organs. Some common complications associated with HCL include:

1. Infections: HCL can weaken the immune system, making individuals more susceptible to infections. Recurrent or severe infections can occur due to decreased normal white blood cells that help fight infections.

2. Anaemia: HCL can cause a reduction in red blood cells, leading to anaemia. Anaemia can result in fatigue, weakness, shortness of breath, and pale skin due to a decrease in the oxygen-carrying capacity of the blood.

3. Bleeding: HCL can lead to low platelet counts, a condition called thrombocytopenia. Platelets are required for proper blood clotting, and low platelet counts can increase the risk of bruising. It may also increase the risk of bleeding from the gums or nose.

4. Splenomegaly: The abnormal accumulation of hairy cells in the spleen can cause it to enlarge, a condition known as splenomegaly.

5. Other cancers: HCL patients may have a slightly high risk of developing other cancers, such as Hodgkin’s and non-Hodgkin’s lymphomas. However, it is unclear if these other cancers are caused by HCL or cancer treatments. 

When to See a Doctor?

It is advisable to consult a doctor if the patient experiences any persistent or concerning symptoms associated with hairy cell leukaemia. Some signs and symptoms that warrant medical attention include:

1. Weakness or fatigue

2. Easy bruising or bleeding

3. Frequent infections

4. Painless lumps in the underarm, neck, stomach, or groin

5. A feeling of fullness below the ribs

6. Shortness of breath

If a person is receiving hairy cell leukaemia treatment, they should see their healthcare professional when symptoms worsen or they experience intense side effects.

There is no specific diet for hairy cell leukaemia, but maintaining a healthy and balanced diet is generally beneficial for overall well-being. It can support the body during and after treatment. Here are some general dietary recommendations to consider:

1. Eat Foods Obtained from Plants: Nutrients from plant sources, like fruits, vegetables, nuts, seeds, etc., are the healthiest way to achieve a healthy weight. 

2. Choose Leaner Proteins: Incorporate lean protein sources such as poultry, fish, legumes, tofu, and low-fat dairy products into the meals. Maintaining muscle is crucial for leukaemia patients who may experience weight loss, and protein plays a key role in building and maintaining muscle.

3. Limit Processed Foods: Cancer patients should minimize the consumption of processed food and red meat. It is recommended to consume less than 0.5 kg of red meat every week. 

4. Limit Alcohol Consumption: Patients must try to quit alcohol consumption. If they consume alcohol, the recommended intake is no more than two glasses daily for men and one for women. 

Some other dietary tips include:

1. Eating small meals every two to three hours

2. Switching to plastic utensils to eliminate the metallic taste

3. Trying ginger or mint products to help with nausea

4. Drinking green tea (it is known to inhibit cancer cell growth)

5. Adding mushrooms and turmeric to the diet due to their anti-inflammatory property

6. Washing fruits and vegetables properly and cooking the food thoroughly

Hairy cell leukaemia is a rare form of blood cancer characterised by the abnormal growth of B cells. It typically presents symptoms such as fatigue, infections, fever, and enlarged spleen.

Timely diagnosis and appropriate treatment, including chemotherapy, targeted therapy, and immunotherapy, can lead to long-term remission and improved quality of life for individuals with HCL. Regular monitoring and follow-up are crucial to detect any complications or potential relapse.

HexaHealth, a comprehensive healthcare platform, can be vital in supporting individuals with HCL. With our vast range of services and resources, we provide access to medical professionals specialising in haematological disorders like HCL.

You can receive expert guidance, obtain second opinions, and access up-to-date information on hairy cell leukaemia pathological outlines, the latest treatments and research. Contact us TODAY to know more!

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