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Cancer that begins in cells of the immune system.

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The drug nivolumab (trade name: Opdivo) has been approved in Germany since November 2016 for the treatment of Hodgkin's lymphoma. It is a treatment option for adults if their cancer returns after stem cell transplantation and antibody treatment with brentuximab vedotin, or if these treatments were not effective enough.

Informed Health Online [Internet] - Institute for Quality and Efficiency in Health Care (IQWiG).

Version: April 20, 2017

Idelalisib (trade name: Zydelig) has been approved in Germany since September 2014 for the treatment of follicular lymphoma or chronic lymphatic leukemia.

Informed Health Online [Internet] - Institute for Quality and Efficiency in Health Care (IQWiG).

Version: January 2, 2015

There is a lack of evidence from randomised controlled trials for the efficacy and safety of different interventions for treating AIDS‐associated Hodgkin's disease, regarding both overall survival and disease‐free survival in treatment‐naive adults with AIDS. Lymphoma is a cancer of the lymphatic system. Lymphomas are classified into two general types: Hodgkin's disease and non‐Hodgkin's lymphomas. Hodgkin's disease is the most common non‐AIDS‐defining malignancy in HIV‐infected patients. Randomised controlled trials for the efficacy and safety of different interventions for treating AIDS‐associated Hodgkin's disease on overall survival and disease‐free survival in treatment‐naive adults with AIDS could not be found.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2011

The objective of this review was to assess the efficacy of the treatment of lymphoma in children with antibody therapy in terms of survival, relapse rates and response to treatment, compared with therapy not including antibody treatment. Furthermore, it aimed to evaluate the effects of antibody therapy on quality of life and side effects.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2016

The drug ibrutinib (trade name: Imbruvica) has been approved in Germany since October 2014 for the treatment of mantle cell lymphoma in adults.

Informed Health Online [Internet] - Institute for Quality and Efficiency in Health Care (IQWiG).

Version: May 2, 2016

Burkitt lymphoma is an important cancer, particularly in children. It is a fast growing tumour but also very sensitive to chemotherapy. It presents a challenge in endemic areas due to late presentation and an often incomplete complement of drugs available for treatment. Different regimens are in use for treatment with varied success rates. This review aims to evaluate these treatments to assess their effectiveness especially for later stages. The review identified 13 trials involving 1824 participants. However, data presentable for the review were only available in 10 trials with 732 participants. The data were difficult to collate because of the quality of the study methods and the reporting of the results; outcome measures differed between trials and they were mainly small‐sized trials. No significant differences in overall survival were seen between studies aimed at inducing remission. Adverse events reported were mostly due to infections and reductions in blood cell counts. The more recent studies were focused on using less intensive treatment regimens as they could provide similar responses with lower risk of adverse effects.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2011

Lymphoma is a cancer of the lymphatic system. There are two general types: Hodgkin's disease (HD) and non‐Hodgkin's lymphoma (NHL). Non‐Hodgkin's lymphoma is the most common AIDS‐defining malignancy in HIV‐infected patients. The most frequent clinical presentations of NHL during AIDS are systemic illness with the compromise of the primary central nervous system and with primary effusion. In people with HIV infection, most lymphomas have originated in an aggressive B‐cell precursor and have a high‐to‐intermediate histology grade. Randomised controlled trials (RCTs) of different interventions for treating AIDS‐associated NHL found unclear evidence for efficacy and safety.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2009

Study design: Meta‐analysis of seven randomised controlled trials involving 1943 patients.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2008

Follicular lymphoma is a B‐cell lymphoma characterised by an initial response to treatment that is usually followed by relapse and progression. Most patients present with advanced disease that cannot be cured. Lymphoma B‐cells express CD20. Rituximab, a monoclonal anti‐CD20 antibody, is expected to be active against cells that express CD20. Compared to chemotherapy alone, rituximab in combination with chemotherapy improves overall survival when used for induction therapy (treatment designed as a first step toward reducing the number of cancer cells) for patients with newly diagnosed or relapsed indolent lymphoma. Clinical trials that have shown improved event‐free survival were inconsistent regarding overall (all‐cause) survival. We aimed to evaluate the effects of maintenance therapy with rituximab on overall survival in patients with follicular lymphoma.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2009

The aim of this systematic review is to outline the possible benefits (i.e. prolonging survival) and also the disadvantages (adverse events) of therapy with interferon‐alpha, administered alone or in combination with other proven drug regimens (otherwise known as chemotherapy) to patients affected by follicular non‐Hodgkin's lymphoma. Interferons are proteins secreted by vertebrate cells that exhibit various biological actions. They confer resistance against many different viruses, inhibit proliferation of normal and malignant cells, augment natural killer cell activity, and show several other immunomodulatory functions. Interferons, types alfa‐2a or alfa‐2b, are usually administered in combination with other drugs to treat a variety of infective and neoplastic diseases. The results showed a significant benefit in progression‐free survival in patients treated with interferon‐alpha alone or combined with chemotherapy as compared with comparator therapies. There was, however, less evidence that interferon‐alpha supported any benefit on overall survival. Furthermore, the presence of relevant drug‐related adverse events suggested that a careful analysis of the risks and benefits has to be performed when making a specific clinical decision about this therapy.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2010

Lymphoma is a cancer that begins in the lymph nodes. It can be treated with chemotherapy (anti‐cancer drugs), but this disrupts the immune system and lowers white cell counts. This can increase a person's risk of infection and limit the amount of chemotherapy that can be given. Granulopoiesis‐stimulating factors (GSF) can increase the body's production of white cells. The review found that treatment with GSF increases white cell counts and reduces the risk of infection in people receiving chemotherapy for lymphoma. However, GSF treatment did not improve survival. More research is needed to improve GSF treatments.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2008

Lymphoma is a cancer that originates from cells of the immune system in the lymph nodes, called lymphocytes. Slow‐growing (indolent) lymphoma is a group of lymphomas characterised by slow and continuous growth, a high initial response rate to treatment that target lymphoma cells (chemotherapy or rituximab), but a relapsing and progressive disease course. It includes follicular lymphoma, small lymphocytic lymphoma and chronic lymphocytic leukaemia, mantle cell lymphoma, lymphoplasmacytic lymphoma and marginal zone lymphoma. With current therapy people with advanced‐stage indolent lymphoma will experience relapse of their disease. Bendamustine is a type of chemotherapy that can be given to people with indolent lymphoma.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2012

Primary cutaneous T‐cell lymphomas (CTCLs) are a subgroup of blood cancers called non‐Hodgkin lymphomas. CTCL is usually seen first in the skin. It is characterised by an uncontrolled increase in T‐lymphocytes, which are a special kind of white blood cell. Most people who get the disease are older than 60 years of age. Compared to other T‐lymphocyte diseases, this type usually progresses slowly. The likely course and outcome of the disease is also better for this type of cancer. However, there is still no cure yet. The most common subtype of CTCL is Mycosis fungoides (MF) which typically grows slowly in the early stages. However, approximately 20% of people in early‐stage of the disease will get worse and progress to tumour stage or develop a type of leukaemia called Sézary syndrome. Most of these people are then subjected to chemotherapies to kill off the cancer cells. The chemotherapy may involve one or more drugs (known as monochemotherapy or polychemotherapies). Although some patients respond well at the beginning of treatment, the disease often returns and life expectancy is uncertain. Furthermore, many patients can experience severe side effects from the treatment. In the last few years, several publications have reported durable responses following a procedure called allogeneic stem cell transplantation (alloSCT). This is when the patient receives a transplant of stem cells from another donor. Before the transplant begins, the patient undergoes treatment to reduce the size of the tumour. This is called full‐intensity or reduced‐intensity conditioning (RIC). Reduced‐intensity conditioning allows the patient to avoid the standard regimes of high‐dose therapy. It appears to be equally effective but with significantly less toxicity. The use of reduced‐intensity conditioning means that older people, who are the majority of patients with this disease, can be treated with stem cells.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

FL is the most common indolent non‐Hodgkin's lymphoma (NHL). It is a slowly progressive disease, with a risk of transformation to a more aggressive lymphoma. Advanced disease (stage III and IV) has been considered incurable. However, in recent years, the use of combination therapy and treatment with the monoclonal anti‐CD20 antibody rituximab, have prolonged survival and decreased transformation rate, and is now considered standard of care in FL patients. One of the most common chemotherapies used in the treatment of FL is the combination of rituximab with cyclophosphamide, vincristine, adriamycin, prednisone (R‐CHOP), which contains an anthracycline (adriamycin). Other anthracycline‐containing regimens (ACR) have also been used to treat FL patients. However, there is no proof that ACRs are better than non‐anthracycline‐containing regimens (non‐ACRs), and there are no standard guidelines for the initial treatment of advanced FL. Importantly, anthracyclines have serious side effects, notably a decrease in blood counts and potential damage to the heart, depending on the dose.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

Primary central nervous system lymphoma (PCNSL) is a malignant disease of the lymphatic system that accounts for about 2% to 5% of all primary intracranial tumours in immunocompetent patients. It is a form of extranodal non‐Hodgkin lymphoma (NHL) and appears at a median age of 62 years. PCNSL is a rare disease with an incidence of 2.7 cases per million population per year, but since the 1990s the occurrence of it has increased in immunocompetent as well as immunocompromised (mostly human immunodeficiency virus (HIV)‐infection related) populations. Symptoms of PCNSL can present manifold though the usual signs are neurological deficits, neuropsychiatric symptoms and raised intracranial pressure. Despite improved treatment strategies, overall survival is still poor and a standard of care for PCNSL patients has not been defined yet. However, high‐dose methotrexate (HD‐MTX) with additional chemotherapy is considered to increase overall survival although the value of additional chemotherapy remains unclear, as there is evidence of a higher risk of adverse events. In this systematic review we summarised and analysed the evidence from randomised controlled trials (RCTs) on efficacy and safety of methotrexate combined with additional chemotherapy in the treatment of adult, immunocompetent PCNSL patients regarding overall survival, progression‐free survival, response rate, adverse events, treatment‐related mortality and quality of life. We searched several important medical databases such as CENTRAL and MEDLINE and found one RCT with 79 patients that fulfilled our inclusion criteria. As a result, this review shows that patients treated with methotrexate plus cytarabine compared to high‐dose methotrexate alone have a statistically significant improvement in progression‐free survival and response rate. No statistically significant difference is shown for overall survival. Adverse events, especially infections, hepatotoxicity and haematological toxicities are more common in patients undergoing therapy with methotrexate plus cytarabine, although there are no differences in terms of treatment‐related mortality. Owing to the small number of included trials and patients, the findings in this review remain uncertain and more RCTs with enlarged numbers of patients and longer follow‐up periods are needed. However, the one analysed study demonstrated that RCTs are feasible on patients with this rare disease and should concentrate on overall survival.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2012

This investigation had 2 aims Determining the patient-relevant benefit of PET and/or PET/CT This was primarily concerned with describing the patient-relevant benefit that doctors and patients can expect from imaging procedures using PET or PET/CT in malignant lymphoma. Its use was considered in the following indications: Determination of the tumour stage (staging), Treatment response of lymphoma (residual disease evaluation/restaging), and Evidence of recurrence in the case of justified suspicion “Benefit” was understood here to mean changes that have perceptible consequences for the patient, such as the effect on mortality and morbidity, the optimum choice of treatment options available with more or less toxic side effects, the general clinical management of the patient and changes in quality of life. Assessing the diagnostic and prognostic accuracy of PET or PET/CT Due to the lack of valid primary trials on determining patient-relevant benefit (first aim), a systematic assessment of the diagnostic and prognostic accuracy of PET or PET/CT was also carried out (second aim). This was primarily concerned with finding out to what extent PET or PET/CT is superior to the standard diagnostic procedure without PET. In other words: Does the use of PET or PET/CT offer an improvement in accurate staging along with the various prognostic consequences, in the accurate recognition of patients with or without residual tumours after treatment is finished, or in the correct diagnosis or correct exclusion of recurrences? In a similar vein, does the use of PET or PET/CT enable more reliable prognostic statements to be made concerning a recurrence than was possible with existing standard diagnostic procedures?

Institute for Quality and Efficiency in Health Care: Executive Summaries [Internet] - Institute for Quality and Efficiency in Health Care (IQWiG).

Version: March 31, 2009

Hodgkin lymphoma (HL) is a malignancy of single lymph nodes, the lymphatic system, and might affect other additional organs. It is a relatively rare disease, accounting for two or three people per 100,000 every year in Western countries, but it is one of the most common cancers in young adults between 20 and 30 years of age. The second peak of the disease is after the age of 60 years. Treatment options for HL have improved since the 1980s, so that even patients in advanced stages may be cured with adequate therapy. Treatment approaches include chemotherapy, radiotherapy or chemotherapy combined with radiotherapy (combined‐modality treatment), of which the combined‐modality treatment is standard for most patients nowadays. Nevertheless, 15% to 20% of patients do not reach complete remission and have refractory disease or relapse. For these patients high‐dose chemotherapy (HDCT) followed by autologous stem cell transplantation (ASCT) has become the optimal treatment option. However, the impact of this regimen on overall survival is still unclear. Therefore, we conducted a Cochrane Review on efficacy and safety of HDCT followed by ASCT in patients with primary refractory or relapsed HL. We searched several important medical databases (the Cochrane Central Register of Controlled Trials and MEDLINE) and summarised and analysed evidence from randomised controlled trials (RCTs). We identified three RCTs corresponding to our pre‐defined inclusion criteria treating 398 patients. We included two trials that compared HDCT followed by ASCT versus conventional chemotherapy alone, and one trial evaluating additional sequential HDCT (SHDCT) followed by ASCT against HDCT followed by ASCT.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

Background: Primary central nervous system lymphoma (PCNSL) is a type of cancer that occurs in the brain or spinal cord. It is a rare and aggressive type of lymphoma. People who develop PCNSL survive for only four months on average, if they do not receive treatment. For a long time the only treatment showing any benefit was whole brain radiotherapy (WBR), in which X‐rays are used to destroy cancerous cells in the brain. However, several studies suggest that this treatment method also produces signs of damage to healthy brain tissue. Since the introduction of methotrexate, a powerful chemotherapy drug showing great beneficial effects, experts have debated the role of radiotherapy in the treatment of people with PCNSL. Radiotherapy could be combined with chemotherapy, or not used at all, especially considering its potentially harmful effects.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Follicular lymphoma is a malignancy of the lymphatic system and a common type of non‐Hodgkin lymphoma. Follicular lymphoma arises from B‐cells, mainly affects older adults and because of its slow growth it is called an indolent lymphoma. Follicular lymphoma grows unnoticed for a long time and is recognised by lymph node enlargement, fever, weight loss, sweating or fatigue. It is called follicular lymphoma because affected lymph nodes show rounded structures called "follicles". Using computer tomography scans, bone marrow biopsy and blood tests, follicular lymphoma is classified into the early Ann Arbor stages I and II or the advanced Ann Arbor stages III or IV, which are diagnosed in the majority of patients. Prognosis and therapy are related to the extent of the disease at initial diagnosis. The small number of patients in stages I or II may be cured by radiotherapy. In advanced stages III or IV, patients are regarded as incurable. Chemotherapy plus the monoclonal antibody rituximab is considered as current treatment strategy for symptomatic patients in advanced stages. Positive effects of high‐dose therapy with transplantation of patients' own stem cells (autologous) are known for patients in advanced stages, especially for the endpoint progression‐free survival. However, this treatment option could have comparatively more treatment‐related late side effects than chemotherapy, including secondary malignancies.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2011

For the treatment of early unfavourable and advanced stage Hodgkin lymphoma (HL) two different international standards are commonly used, either chemotherapy with escalated (intensified) BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) regimen or chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) regimen.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: May 25, 2017

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