Understanding Hairy Cell Leukaemia: Symptoms, Causes, Treatment & Survival Rate

Among the many forms of blood cancer, hairy cell leukaemia stands out for one unusual feature: malignant B-cells covered with tiny, hair-like projections that change how the immune system works. It is a slow-developing and rare blood cancer affecting mainly the marrow and blood. It is not the most common form of leukaemia because it is only associated with approximately 2% of all cases. Still, it is unique in its nature, and the response rate to treatment is also very high.

To truly understand this rare disease, this step-by-step guide will familiarise you with all the essential information, including hairy cell leukaemia and its initial manifestations, causes, diagnosis and treatment options.

What is Hairy Cell leukaemia?

HCL is a rare form of chronic B-cell leukaemia that develops in the bone marrow. The name of the disease is associated with the appearance of the projections on the surface of abnormal B-lymphocytes, which resemble hair under the microscope. These mutated cells grow at a sluggish pace, crowding out normal blood-forming cells and disrupting the normal functioning of the immune system.

Hairy cell leukaemia causes a low red blood cell, white blood cell, and platelet count, which in turn causes fatigue, frequent infections, and easy bruising. HCL has a slow progression compared to aggressive leukaemias, and thus provides the ability to manage the disease over a long period and respond well to therapy.

Hairy Cell Leukaemia Causes

The exact cause of HCL is unclear, but researchers have shown that genetic mutations in the B-cells may cause it. The BRAF V600E mutation is one of the most widespread mutations associated with and leading to uncontrolled cell growth.

The possible causative agents may include:

• Radiation or exposure to toxic chemicals in the environment.
• Hereditary predisposition or family medical history of leukaemia.
• Malfunctions in the immune system often cause abnormal cell proliferation.

However, there is no specific reason that directly explains why some individuals develop HCL, and current studies are still trying to unravel the underlying biological processes.

Hairy Cell Leukaemia Symptoms

The early Hairy Cell leukaemia symptoms are usually overlooked. The majority of the patients are diagnosed upon discovery of abnormalities in routine blood tests.

Common Hairy Cell leukaemia symptoms include:

• Anaemia results in fatigue and weakness.
• Recurrent infections due to a low white blood cell count.
• Low platelet levels result in easy bruising or bleeding.
• Splenomegaly, or an enlarged spleen, can lead to abdominal discomfort or fullness.
• Loss of weight, unexplained.

The symptoms take a long time to develop, and therefore some patients can spend years living with the disease before they can be treated.

Hairy Cell Leukaemia Diagnosis

Hairy cell leukaemia diagnosis requires several laboratory and radiographic examinations. The process of hairy cell leukaemia diagnosis encompasses:

• Blood tests- to test the red blood cells, white blood cells, and platelets. The low number is likely to be a sign of a bone marrow disorder.
• Peripheral blood smear- a microscope observation identifies the presence of the so-called hairy B-cells.
• Bone marrow biopsy- this is done to ascertain the diagnosis and also to determine the extent of bone marrow involvement.
• Morphologic and Immunophenotypic Features- assist in the detection of particular markers of cells (CD11c, CD25, CD103, CD123).
• Genetic testing- determines the presence of the BRAF V600E mutation, which contributes to the right diagnosis.

Early and proper diagnosis is also essential in order to implement an optimal treatment regimen in the case of hairy cell leukaemia.

Hairy Cell Leukaemia Treatment

Asymptomatic patients with stable blood counts do not normally need treatment right after diagnosis. Nevertheless, as soon as the symptoms manifest or the number of blood cells decreases significantly, treatment starts. The common options of hairy cell leukaemia treatment are:

1. Chemotherapy

• The most widely used drugs are cladribine (2-CdA) and Pentostatin (DCF).
• These drugs are specific to abnormally dividing cells, such as HCL, and healthy cells can multiply.
• One course of treatment can often result in several years of long-term remission.

2. Targeted therapy

• BRAF V600E Vemurafenib is a BRAF inhibitor prescribed for patients with the BRAF V600E mutation who have relapsed or are resistant to chemotherapy.
• It specifically suppresses the activity of the mutated gene in cancer cells and decreases the growth of cancerous cells.

3. Immunotherapy

• Rituximab is a monoclonal antibody that helps the immune system identify and eliminate leukaemia cells.
• It is either used independently or with chemotherapy to achieve enhanced results.

4. Splenectomy (Surgery)

• Surgery could be advised in exceptional situations when the spleen is over-enlarged or produces pain. But this technique is less frequently used in successful medical care.

5. Supportive Care

• Preventing infection with antibiotics.
• Transfusion of blood on demand.
• Nutrition to improve general health.

The modern treatment has transformed the survival rates and made HCL one of the most curable leukaemias.

Hairy Cell Leukaemia Survival Rate

The hairy cell leukaemia survival rate is very high. With proper treatment:

• Approximately 95 per cent of the patients are brought into total remission.
• The 5-year survival is above 90, and most of them live for decades after the treatment.
• The disease is normally responsive to retreats even when it has relapsed.

HCL is among the most manageable chronic blood cancers with such an impressive survival rate.

Hairy Cell Leukaemia Life Expectancy 

With the current medical progress, the hairy cell leukemia life expectancy of patients with hairy cell leukaemia is almost normal. With early diagnosis and the usual treatment, the patient will be able to live 20 years or longer. Long-term remission is maintained through regular monitoring, a healthy lifestyle, and adherence to treatment guidelines.

Can Hairy Cell Leukaemia be Cured?

Although doctors seldom discuss the cure of leukaemias, HCL may be subjected to decades of control. Several patients get full remission following a single course of treatment, and others do not get another relapse. In infrequent instances of a relapse, newer targeted treatments like vemurafenib or immunotherapy remit the condition once more.

Therefore, although it is not always curable, HCL is very treatable and can have an almost normal life expectancy.

Living with Hairy Cell Leukaemia

HCL patients need frequent follow-ups, blood tests, and periodic bone marrow assessments. Most people lead productive and fulfilling lives with minimal disruptions, as the disease has a slow progression. A healthy immune system, regular exercise, and effective stress management are crucial factors for overall well-being.

Emotional support groups and medical counselling serve the purpose of helping patients deal with the mental strain that comes along with the diagnosis of cancer, too.

Conclusion

Hairy cell leukaemia is a blood cancer that is rare yet very curable. This is because early detection, accurate Hairy cell leukaemia diagnosis, and early intervention of the disease assure the vast majority of patients a long and healthy life. The prognosis of HCL is most encouraging as the medical treatment and treatment options continue to improve.

In the case of an HCL patient (you or someone acquainted with one), emphasise balanced nutrition, immune support, and regular medical check-ups.

Frequently Asked Questions Related to Hairy Cell Leukaemia