Hairy Cell Leukaemia Recurrent

Hairy cell leukaemia is a rare type of blood cancer that often comes back after treatment, but patients can achieve further periods of remission with repeated therapy.

Table of contents

• What is Recurrent Hairy Cell Leukaemia?
• Treatment Options for Relapsed Disease
• How Treatment is Given
• Treatment Outcomes and Response Rates
• Other Treatment Approaches
• Coping with Relapse

What is Recurrent Hairy Cell Leukaemia?

When hairy cell leukaemia comes back after treatment, it is called a relapse or recurrent disease^[4]. You might need further treatment if tests show that your hairy cell leukaemia has not responded to first line treatment, or if the leukaemia returns after a period of time^[4].

The time between remission (when cancer symptoms disappear) and relapse varies from person to person. Some people have months without symptoms and others have years^[4]. Around 50% of people in hairy cell leukaemia remission survive without relapse for ten years^[5].

Patients with two or more relapses experience frequent further relapses, with increasingly shorter time to next treatment as the number of treatment lines increases^[14]. While 30 to 40 percent of patients will relapse after initial treatment, you can have a further remission with more treatment^[4][15].

Treatment Options for Relapsed Disease

Second line treatment is usually chemotherapy (medicines that kill cancer cells) again^[4]. You might have chemotherapy on its own or combined with a targeted cancer drug (medicine that targets specific features of cancer cells)^[4].

Most people have chemotherapy as a first treatment for hairy cell leukaemia. You usually have either cladribine or pentostatin^[4]. The second line treatment depends on the time since your last treatment, what treatment you have already had, what clinical trials are available, and your general health and symptoms^[4].

You might have the same chemotherapy drug again. This means that if you had cladribine treatment the first time, you might have it again. Or you might have pentostatin as your second line treatment^[4]. You either have chemotherapy on its own, or combined with the targeted cancer drug called rituximab^[4].

There are other treatment options if you relapse again. These include a different chemotherapy (such as bendamustine) combined with rituximab, or clinical trials looking at different targeted drugs^[4].

Patients with relapsed or refractory hairy cell leukemia who were treated with vemurafenib experienced excellent response and relapse-free survival, according to a recent clinical trial. Vemurafenib is a small molecular inhibitor drug that blocks BRAF V600E, a key genetic mutation in hairy cell leukaemia cells that promotes cancer cell survival^[15]. Of these patients, 33 percent had a complete response to the drug and 53 percent of patients had a partial response^[15].

How Treatment is Given

How you have treatment depends on which treatment you receive. Treatment can be an injection under the skin or an injection into a vein as a drip^[4].

You usually have injections under the skin into the stomach, thigh or top of your arm. You might have stinging or a dull ache for a short time after this type of injection but they don’t usually hurt much. The skin in the area may go red and itchy for a while^[4].

For treatment into the blood stream, you have the treatment through a drip into your arm. A nurse puts a small tube (a cannula) into one of your veins and connects the drip to it^[4].

Treatment Outcomes and Response Rates

In studies of patients with two or more relapses, the overall blood response rate after third line treatment was 97% (complete response 86%) with a 40% five-year chance of relapse. The average time without relapse was 104 months, and average survival was 235 months^[14]. After fourth line treatment, the overall blood response rate was 94% with a 27% two-year chance of relapse^[14].

In vemurafenib studies, a 40-month follow-up evaluation revealed that 68 percent of patients experienced relapse, with an average relapse-free survival rate of 19 months. Of the 21 patients who relapsed, 14 were re-treated with vemurafenib and of these patients, 86 percent had their white blood cell levels return to normal^[15]. Overall, patient survival was 82 percent at four years^[15].

The duration of remission was shorter with each subsequent relapse^[15]. Increasing drug dosage or extending treatment duration did not improve the overall treatment response^[15].

Other Treatment Approaches

You might have other treatments to treat symptoms of hairy cell leukaemia, or to prevent problems caused by the leukaemia. This can include surgery or supportive treatments such as antibiotics or blood products^[4].

Surgery is not a common treatment for hairy cell leukaemia. But rarely, the doctor might suggest an operation to remove the spleen. Removing the spleen can help with some of the symptoms caused by a swollen spleen^[4].

Hairy cell leukaemia and its treatment can cause problems. Supportive treatments can help to either prevent or control these problems. Supportive treatments include preventing and treating infections, and giving blood products^[4].

Doctors and researchers do trials to make existing treatments better and develop new treatments. As hairy cell leukaemia is rare, there are fewer trials, compared to some types of cancer. There may not always be a trial available or suitable for your situation. But do discuss this with your specialist if you’re interested in taking part in a trial^[4].

Coping with Relapse

Hairy cell leukaemia can be difficult to cope with. Knowing that it is going to come back at some point but not knowing when is especially difficult^[4]. Living with uncertainty is hard. And when it does come back it can feel very difficult even when you have known it will happen^[4].

The type of support people need varies. Finding what works for you is important. Talking to family and friends helps many people^[4]. Patients with recurrent disease often benefit from staying positive and believing they can get through treatment again. Many patients report that despite the challenges, life can return to normal and be lived to the fullest after treatment^[18].

Some patients find it helpful to remember that if you have to have a leukaemia, this is one of the better types to have, as treatment is highly successful. The quiet reflection that comes from enduring treatment can bring personal growth in the long run^[18].