ATLL Treatment
Treatment for ATLL varies depending on the sub-type and stage of disease as well as geography. Basically different countries have different treatment guidelines for the different ATLL subgroups.
Hematopoietic stem cell transplantation (HSCT)
ATLL is potentially curable. This is when the bone marrow is completely depleted of all its cells. This way all infected cells in the bone marrow and in the blood are destroyed. New cells need to be donated to the patient so that the bone marrow is repopulated with healthy non-cancer blood cells.
It can be dangerous to ‘wipe out’ the bone marrow with chemo and radiotherapy, since for a period of time the immune system is out of function and patients can catch serious infections.
A suitable bone marrow donor might be difficult to find, since the blood cells of the donor must match the patients so that the transplant is accepted. This is called HLA matching. Usually close family members are asked to be tested for HLA matching. Still some patients develop side-effects to the transplantation, one of which is called graft-versus host reaction. Sadly for most patients this intensive and expensive treatment might be difficult or impossible to access. Some patients might not be fit enough to receive this aggressive treatment.
However until a suitable donor is available time can be bought with the following treatment:
Hematopoietic stem cell transplantation (HSCT)
ATLL is potentially curable. This is when the bone marrow is completely depleted of all its cells. This way all infected cells in the bone marrow and in the blood are destroyed. New cells need to be donated to the patient so that the bone marrow is repopulated with healthy non-cancer blood cells.
It can be dangerous to ‘wipe out’ the bone marrow with chemo and radiotherapy, since for a period of time the immune system is out of function and patients can catch serious infections.
A suitable bone marrow donor might be difficult to find, since the blood cells of the donor must match the patients so that the transplant is accepted. This is called HLA matching. Usually close family members are asked to be tested for HLA matching. Still some patients develop side-effects to the transplantation, one of which is called graft-versus host reaction. Sadly for most patients this intensive and expensive treatment might be difficult or impossible to access. Some patients might not be fit enough to receive this aggressive treatment.
However until a suitable donor is available time can be bought with the following treatment:
Acute ATLL
The first line therapy is Interferon (IFN) and zidovudine (AZT). It has shown to be superior to chemotherapy alone. A very important research project summarised all treatment data for ATLL living in France, the USA, the Caribbean and England:
In the UK all patients with acute ATLL receive IFN-alpha + AZT alone as first line therapy for two weeks and then reassessed for response.
Only if disease deems to be progressing will chemotherapy (CHOP) be added in.
IFN-alpha + AZT is continued indefinitely if the patient responds well to the treatment.
The first line therapy is Interferon (IFN) and zidovudine (AZT). It has shown to be superior to chemotherapy alone. A very important research project summarised all treatment data for ATLL living in France, the USA, the Caribbean and England:
- IFN-alpha + AZT reached similar complete response rates (35%) to chemotherapy (CHOP) alone (25%)
- 5 year survival increased from 10% if given chemotherapy to 28% if given IFN-alpha + AZT alone
- Adding IFN-alpha + AZT to chemotherapy did not improve outcomes
- In patients with complete remission IFN-alpha + AZT alone resulted in much better survival (5 year, 82%)
In the UK all patients with acute ATLL receive IFN-alpha + AZT alone as first line therapy for two weeks and then reassessed for response.
Only if disease deems to be progressing will chemotherapy (CHOP) be added in.
IFN-alpha + AZT is continued indefinitely if the patient responds well to the treatment.
Lymphomatous ATLL
In the UK patients are usually offered 2-3 cycles of chemotherapy (CHOP) to reduce the size of the tumours. This is when Interferon-alpha (IFN-alpha) and zidovudine (AZT) are added in while the chemotherapy continues.
This early addition of IFN-alpha + AZT is associated with a significant improvement in 5 year survival, up to 20%, compared with chemotherapy alone.
IFN-alpha + AZT are continued indefinitely if the patient has responded well to the treatment.
Chronic and Smoldering ATLL (indolent ATLL)
Patients who received first-line IFN-alpha + AZT therapy only had an excellent survival: 100% survived beyond 5 years. But only 42% of patients who received first-line chemotherapy (CHOP), with or without IFN-alpha + AZT therapy reached 5 year survival. These patients often do not need a HSCT.
IFN-alpha + AZT are continued indefinitely if the patient has responded well to the treatment.
Monitoring
Treatment response and drug side-effects are monitored closely by the medical team through examination, blood tests and CT or MRI scans.
Covering for Opportunistic Infections
Patients with ATLL have a weaker immune-system. The immune-system becomes even more impaired when treatment is started. Doctors may wish to cover the patient with additional antibiotic and antiviral medication to prevent infections occurring.
Pneumocystis jerovecii pneumonia prophylaxis
Herpes simplex prophylaxis
Strongyloides stercoralis
CMV treatment should be initiated if the CMV viral load is rising
References:
Hodson A, Crichton S, Montoto S, Mir N, Matutes E, Cwynarski K, Kumaran T, Ardeshna KM, Pagliuca A, Taylor GP, Fields PA.Use of zidovudine and interferon alfa with chemotherapy improves survival in both acute and lymphoma subtypes of adult T-cell leukemia/lymphoma. J Clin Oncol. 2011 Dec 10;29(35):4696-701.
Bazarbachi A, Plumelle Y, Carlos Ramos J, Tortevoye P, Otrock Z, Taylor G, Gessain A, Harrington W, Panelatti G, Hermine O. Meta-analysis on the use of zidovudine and interferon-alfa in adult T-cell leukemia/lymphoma showing improved survival in the leukemic subtypes. J Clin Oncol. 2010 Sep 20;28(27):4177-83. doi: 10.1200/JCO.2010.28.0669.
In the UK patients are usually offered 2-3 cycles of chemotherapy (CHOP) to reduce the size of the tumours. This is when Interferon-alpha (IFN-alpha) and zidovudine (AZT) are added in while the chemotherapy continues.
This early addition of IFN-alpha + AZT is associated with a significant improvement in 5 year survival, up to 20%, compared with chemotherapy alone.
IFN-alpha + AZT are continued indefinitely if the patient has responded well to the treatment.
Chronic and Smoldering ATLL (indolent ATLL)
Patients who received first-line IFN-alpha + AZT therapy only had an excellent survival: 100% survived beyond 5 years. But only 42% of patients who received first-line chemotherapy (CHOP), with or without IFN-alpha + AZT therapy reached 5 year survival. These patients often do not need a HSCT.
IFN-alpha + AZT are continued indefinitely if the patient has responded well to the treatment.
Monitoring
Treatment response and drug side-effects are monitored closely by the medical team through examination, blood tests and CT or MRI scans.
Covering for Opportunistic Infections
Patients with ATLL have a weaker immune-system. The immune-system becomes even more impaired when treatment is started. Doctors may wish to cover the patient with additional antibiotic and antiviral medication to prevent infections occurring.
Pneumocystis jerovecii pneumonia prophylaxis
- Dapsone
- Co-trimoxazole
- Pentamidine
- Atovaquone
Herpes simplex prophylaxis
- Aciclovir Cryptococcosis and/or Candidiasis
- Fluconazole
Strongyloides stercoralis
- Ivermectin and Albendazole
CMV treatment should be initiated if the CMV viral load is rising
- Gancivolvir
References:
Hodson A, Crichton S, Montoto S, Mir N, Matutes E, Cwynarski K, Kumaran T, Ardeshna KM, Pagliuca A, Taylor GP, Fields PA.Use of zidovudine and interferon alfa with chemotherapy improves survival in both acute and lymphoma subtypes of adult T-cell leukemia/lymphoma. J Clin Oncol. 2011 Dec 10;29(35):4696-701.
Bazarbachi A, Plumelle Y, Carlos Ramos J, Tortevoye P, Otrock Z, Taylor G, Gessain A, Harrington W, Panelatti G, Hermine O. Meta-analysis on the use of zidovudine and interferon-alfa in adult T-cell leukemia/lymphoma showing improved survival in the leukemic subtypes. J Clin Oncol. 2010 Sep 20;28(27):4177-83. doi: 10.1200/JCO.2010.28.0669.