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A malignant disease characterized by enlargement of the lymph nodes, spleen, and general lymphoid tissue.

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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: June 20, 2013

Hodgkin lymphoma (HL) is a malignant disease of the lymphatic system of the body. It accounts for 10% to 15% of all lymphoma in industrialised countries and tends to show two peaks in incidence at around 30 and 60 years of age. While it is considered a relatively rare disease, it is one of the most common malignancies in young adults. With cure rates of up to 90% over 5 years, it is one of the most curable cancers worldwide.

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

Version: January 9, 2015

Hodgkin lymphoma (HL) is a malignancy of the lymphatic system. It occurs in children and adults, but it is more common in the third decade of life. It is one of the most curable forms of cancer. There are four stages of HL, stages I and II are considered as early stage HL and stages III and IV as advanced stage. Using risk factors such as presence or absence of bulky disease and presence or absence of B‐symptoms, like night sweats or fever, early stage HL is further classified into early favourable and early unfavourable stages. Treatment options are chemotherapy, radiotherapy or both. Radiotherapy may have, more treatment‐ related side effects than chemotherapy, including second malignancies; this applies at least to the large treatment fields used in the past. However, with modern, very limited treatment fields, the risks of long‐term side effects caused by radiotherapy have been reduced significantly.

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

Version: April 27, 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

Aggressive Non‐Hodgkin's Lymphomas (NHLs) are fast growing forms of lymphoma. The most common type is a diffuse large B‐Cell lymphoma (DLCL) but there are several other subtypes of aggressive lymphoma and variants of DLCL, such as centroblastic, immunoblastic or anaplastic large B‐Cell lymphoma. Aggressive NHLs are generally responsive to conventional cancer therapies such as chemotherapy and radiation therapy. In the 1980s, many researchers reported that some patients with diffuse, large‐cell lymphoma, who had not responded to conventional chemotherapy, could be cured with high‐dose chemotherapy and autologous stem cell or bone marrow transplantation. This techniques may be used to treat the cancer, because the high doses of chemotherapy can destroy the patient's bone marrow. Therefore stem cells or marrow is taken from the patient before treatment. The marrow or the stem cells are then frozen, and the patient is given high‐dose chemotherapy with or without radiation therapy to treat the cancer. The marrow or the stem cells that were taken out is then thawed and given back through a needle in a vein to replace the marrow that was destroyed. This type of transplant is called an autologous transplant. If the marrow given is taken from another person, the transplant is called an allogeneic transplant.On the first decade of study into autologous transplantation for the treatment of aggressive lymphoma, the focus was on the use of this approach to rescue patients after relapse or if the disease already progressed under standard chemotherapy. These encouraging results in relapsed or progressive lymphoma led to the testing of the technique as a primary therapy for the disease. However, it was also important to identify factors that could predict outcome of the therapy for patients with aggressive lymphoma. The International Prognostic Index score (IPI) was established in 1993. This score was designed to better predict outcome of aggressive lymphoma. Based on the number of negative prognostic factors present at the time of diagnosis (age >60 years, stage III/IV disease, elevated lactate dehydrogenase [LDH] level, Eastern Cooperative Oncology Group [ECOG] performance status > 2, more than one extranodal site of disease) four outcome groups (low‐risk, low‐intermediate risk, high‐intermediate risk and high‐risk ) were identified with a 5‐year overall survival ranging from 26% to 73%.

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

Version: January 23, 2008

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

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: April 18, 2007

We aimed to compare various forms of treatment for newly diagnosed Hodgkin lymphoma involving chemotherapy with or without additional radiotherapy. We particularly looked at the risk of second cancers caused by these treatments, although survival and elimination of Hodgkin lymphoma were also investigated.

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

Version: September 13, 2017

Adult Hodgkin lymphoma treatment depends on the type (classical or nodular lymphocyte predominant) and includes chemotherapy and/or radiation therapy. Get detailed information about the prognosis, and treatment of newly diagnosed and recurrent Hodgkin lymphoma in this expert-reviewed summary.

PDQ Cancer Information Summaries [Internet] - National Cancer Institute (US).

Version: June 1, 2018

Childhood Hodgkin lymphoma (classical or nodular lymphocyte-predominant HL) treatment usually involves chemotherapy and/or radiation therapy. Learn more about the symptoms, diagnosis, prognosis, staging, and treatment of in this expert-reviewed summary.

PDQ Cancer Information Summaries [Internet] - National Cancer Institute (US).

Version: May 31, 2018

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: January 20, 2010

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: July 8, 2009

Hodgkin's disease (HD) patients are usually treated initially with radiotherapy alone (RT; early stages only), chemotherapy alone (CT) or combined chemo‐radiotherapy (CRT). A meta‐analysis of data from 37 randomised trials including over 9000 patients was conducted. For early‐stage patients, CRT resulted in longer survival and longer HD‐free survival than either RT or CT alone. Second malignancy (SM) risk was lower with CRT than with RT (no difference in between CRT and CT was demonstrated). For advanced stages, no difference in survival between CRT and CT was established. With CRT, HD‐free survival was longer but SM risk was higher.

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

Version: October 19, 2005

Non-Hodgkin lymphoma (NHL) options include chemotherapy, radiation, targeted therapy, plasmapheresis, surveillance, stem cell transplant, and surgery. Learn more about types of NHL and treatments in this expert-reviewed summary.

PDQ Cancer Information Summaries [Internet] - National Cancer Institute (US).

Version: June 1, 2018

Childhood non-Hodgkin lymphoma treatment can include chemotherapy, radiation therapy, targeted therapy, and high-dose chemotherapy with stem cell transplant. Learn more in this expert-reviewed summary.

PDQ Cancer Information Summaries [Internet] - National Cancer Institute (US).

Version: May 31, 2018

Blood and lymph node cancers are referred to as haematological malignancies. These are types of cancer that affect the blood, bone marrow and other parts of the lymphatic system. The most common ones are lymphomas, leukaemias and myelomas. Depending on the kind of cancer and how far it has spread, there are a lot of different options to manage the disease. Usually chemotherapy, radiotherapy or a combination of both is used to treat the disease. If the cancer is widespread, a transplantation of the patients’ own bone marrow cells combined with an aggressive chemotherapy can be a treatment option.

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

Version: June 12, 2014

Cancer-related post-traumatic stress can occur any time from diagnosis to after treatment ends. Shock, fear, helplessness, or horror can be felt by cancer patients and lead to cancer-related post-traumatic stress. Learn about the causes and ways doctors can help manage these symptoms of distress in this expert-reviewed summary.

PDQ Cancer Information Summaries [Internet] - National Cancer Institute (US).

Version: July 7, 2015

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: November 14, 2012

Grief, bereavement (mourning), and coping with loss are common challenges following the loss of a loved one from cancer. Learn about coping and the grief process in adults and children in this expert-reviewed information summary.

PDQ Cancer Information Summaries [Internet] - National Cancer Institute (US).

Version: March 6, 2013

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: August 29, 2013

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