Treatment types for B-cell Lymphocytic and Hairy Cell Leukemia

“Standard to care” refers to the best-known treatment. In cancer care, different doctors work together to bring out an overall treatment plan for the patient. This is called a multidisciplinary team. 

Treatments recommendations depend on many factors:

  • The size, grade and type of tumour
  • Whether the tumour is applying pressure on vital parts of the brain
  • If the tumour has increased to other parts of the body
  • Possible side effects
  • The patient’s preferences and overall health

Watch and wait or active surveillance

Most patients may not require treatment for years post-diagnosis. It is suggested that the disease be monitored carefully without active treatment. This is known as ‘watch and wait’ or active surveillance. This approach can be hard to understand, which can be stressful. However, patients can find that their concerns about this approach lessen over time. Talk to the health care team if you have questions or concerns.

During this time, if leukaemia shows signs of worsening, active treatment would then begin. 

Chemotherapy

Chemotherapy uses medicine to kill or stop the growth of cancerous cells. Depending upon the stage, different chemotherapy is given. The point that makes a difference is how the chemotherapy enters the body and which cells it affects.

Systemic chemotherapy enters the bloodstream to reach cancerous cells all over the body.

A chemotherapy schedule usually consists of a certain number of cycles over a fixed period. A patient can be given one drug at a time or a combination of different drugs given simultaneously.

Drugs used to treat HCL include pentostatin (Nipent) and cladribine (Leustatin). These are the standard initial treatment for HCL. For most people having HCL, both drugs can bring a remission that lasts for many years.

PLL is treated with drug combinations, and several different regimens can be used. A specific regimen is suggested for each patient. It depends on the type of chemotherapy the patient may have previously received and the possible side effects.

The side effects depend on the patient and the dose used. Still, they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhoea. These side effects usually go after treatment is completed.

Immunotherapy

Immunotherapy, a type of biological therapy, uses artificial or natural substances to harness our immune system to fight. 

It uses materials formed either by the body or in a laboratory to improve, target, or restore immune system function. Immunotherapy for HCL includes recombinant interferon-alpha (Alferon, Intron A, Roferon-A).

Different types of immunotherapy can cause various side effects. Talk to the doctor about the possible side effects of the immunotherapy recommended for you.

Targeted therapy

Targeted therapy is a treatment that targets leukaemia’s specific proteins, genes, or the tissue environment that contributes to growth and survival. This type of treatment stops the growth and spread of leukaemia cells while limiting damage to normal healthy cells.

Recent studies showed that not all cancers have the same targets. To determine the most effective treatment, the doctor may run tests to identify the genes, proteins, and other factors involved in your leukaemia. This helps doctors better match each patient with the most effective treatment whenever possible. Additionally, many research studies are going on now to discover more about specific molecular targets and new treatments directed at them. 

A monoclonal antibody is a type of targeted therapy. It recognizes and attaches to a specific protein in the leukaemia cells, and it doesn’t affect cells that do not have that protein. Rituximab (Rituxan) is an antibody directed against the CD20, a surface protein. BL22 immunotoxin is an antibody designed to attach to the surface protein CD22 and deliver a toxin to the leukaemia cell. Both BL22 and rituximab have been used to treat HCL that did not go into remission after the initial treatment. However, BL22 is not approved by the FDA for PLL treatment and is available only in clinical trials. Alemtuzumab (Campath) is an antibody that targets the surface protein CD52 and has been used to treat PLL. 

Surgery

The spleen helps form the body’s white blood cells. Surgery to remove the spleen is known as splenectomy. This may be suggested for some patients. 

Before surgery, talk to the health care team about the possible side effects of the specific surgery.

Stem cell/Bone Marrow Transplantation

A stem cell transplant is a medical process in which bone marrow that contains the cancer is replaced by highly specialized cells. These cells, known as hematopoietic stem cells, develop into the healthy bone marrow. Hematopoietic stem cells are the blood-forming cells found in the bloodstream and the bone marrow. These stem cells form all of the healthy cells in the blood. Presently, this procedure is more commonly called a stem cell transplant than a bone marrow transplant because it is the stem cells in the blood typically being transplanted, not the actual bone marrow tissue.

Before suggesting transplantation, doctors talk to the patient and family members regarding the risks of this treatment. They will also consider several other factors, like age and general health, cancer type, and previous treatment results. It is not used often for people with PLL and HCL because it is not always an effective therapy for these diseases and because many patients with PLL or HCL are older and the risks of doing the procedure are higher.

There are two types of stem cell transplantation depending on the source of the replacement blood stem cells – autologous (AUTO) and allogeneic (ALLO). AUTO uses the patient’s stem cells, while ALLO uses donated stem cells. In both types, the target is to destroy all cancer cells in the blood, marrow, and other body parts using high doses of chemotherapy or radiation therapy and then allow replacement blood stem cells to create healthy bone marrow. ALLO transplantation is occasionally considered for younger patients with PLL when chemotherapy is ineffective. However, AUTO transplantation is rarely considered for people having PLL or HCL.

Side effects depend on the child’s general health, the type of transplant, and other factors.

Palliative Care 

Cancer and its treatment have side effects that can be mental, physical or financial and managing the effects are palliative or supportive care.

Palliative care includes medication, nutritional changes, emotional and spiritual support and other relaxation therapies. 

Palliative care focuses on alleviating how you feel during treatment by managing symptoms and supporting patients and their families with other non-medical needs. Regardless of type and stage of Cancer age, any person may receive this type of care.

Refractory HCL or PLL

If HCL or PLL worsen during treatment, it is called refractory or resistant leukaemia. For HCL, the treatment plan may include rituximab or BL22 immunotoxin. Palliative care will also be essential to help relieve symptoms and side effects. Additionally, it has recently been shown that almost all patients having HCL have a mutation in the leukaemia cells in a gene known as BRAF. A drug that blocks a mutated BRAF gene from helping leukaemia grow and spread, known as vemurafenib (Zelboraf), has been of help in treating melanoma. A few patients having refractory HCL have also received this drug. 

Remission and chance of recurrence

When cancer can’t be detected in the body and there are no symptoms, this is known as remission. This may be called having ‘no evidence of disease’.

A remission can be temporary or permanent. Many people worry about the recurrence of cancer.

The doctor performs another round of tests to know the extent of the recurrence.

Mainly the treatment plan includes the treatments explained above, like surgery, chemotherapy, radiation therapy, and targeted therapy.

For example, cladribine and pentostatin can work again to treat HCL if it returns after a long period of remission. The doctor can suggest clinical trials studying new ways to treat recurrent PLL or HCL. 

If treatment doesn’t work

If cancer can’t be treated or controlled, it leads to advanced or terminal cancer. It is vital to have straightforward conversations with your health care team to express your feelings, preferences, and concerns. The health care team has unique skills, knowledge, and experience to assist patients and their families. Ensuring that a person is physically comfortable, free from pain, and emotionally supported is extremely important.