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Risk factors for gastric lymphoma include the following:
- "Helicobacter pylori"
- Long-term immunosuppressant drug therapy
- HIV infection
Of all cancers involving the same class of blood cell, 8% of cases are MALT lymphomas.
Most people affected by primary gastric lymphoma are over 60 years old. Symptoms include epigastric pain, early satiety, fatigue and weight loss.
The factors of poor prognosis for patients with thyroid lymphoma are advanced stage of the tumor, large size (>10 cm) as well as spreading to mediastinum. The overall survival for primary thyroid lymphoma is 50% to 70%, ranging from 80% in stage IE to less than 36% in stage IIE and IVE in 5 years.
Risk factors for pancreatic adenocarcinoma include:
- Age, gender, and ethnicity; the risk of developing pancreatic cancer increases with age. Most cases occur after age 65, while cases before age 40 are uncommon. The disease is slightly more common in men than women, and in the United States is over 1.5 times more common in African Americans, though incidence in Africa is low.
- Cigarette smoking is the best-established avoidable risk factor for pancreatic cancer, approximately doubling risk among long-term smokers, the risk increasing with the number of cigarettes smoked and the years of smoking. The risk declines slowly after smoking cessation, taking some 20 years to return to almost that of non-smokers.
- Obesity; a BMI greater than 35 increases relative risk by about half.
- Family history; 5–10% of pancreatic cancer cases have an inherited component, where people have a family history of pancreatic cancer. The risk escalates greatly if more than one first-degree relative had the disease, and more modestly if they developed it before the age of 50. Most of the genes involved have not been identified. Hereditary pancreatitis gives a greatly increased lifetime risk of pancreatic cancer of 30–40% to the age of 70. Screening for early pancreatic cancer may be offered to individuals with hereditary pancreatitis on a research basis. Some people may choose to have their pancreas surgically removed to prevent cancer developing in the future.
- Chronic pancreatitis appears to almost triple risk, and as with diabetes, new-onset pancreatitis may be a symptom of a tumor. The risk of pancreatic cancer in individuals with familial pancreatitis is particularly high.
- Diabetes mellitus is a risk factor for pancreatic cancer and (as noted in the Signs and symptoms section) new-onset diabetes may also be an early sign of the disease. People who have been diagnosed with Type 2 diabetes for longer than ten years may have a 50% increased risk, as compared with non-diabetics.
- Specific types of food (as distinct from obesity) have not been clearly shown to increase the risk of pancreatic cancer. Dietary factors for which there is some evidence of slightly increased risk include processed meat, red meat, and meat cooked at very high temperatures (e.g. by frying, broiling or barbecuing).
Of all cancers involving the same class of blood cell (lymphoproliferative disorders), 22% of cases are follicular lymphomas.
There is no known cause for any type of Marginal Zone non-Hodgkins lymphoma, but it occurs when the body produces large amounts of abnormal lymphocytes.
Factors that may increase an individuals chance of developing nodal MZL are being over the age of 60 and having been infected with hepatitis C virus. Factors that may increase an individuals chance of developing MALT lymphoma include being over the age of 50, having an autoimmune condition (rheumatoid arthritis, Hashimoto's thyroiditis), and long lasting chronic inflammation due to infection (H.pylori, Sjogren syndrome, chlamidia infection, Borrelia infection, Campylobacter jejuni infection). Factors that increase an individuals risk of developing splenic MZL include the hepatitis C virus, Epstein-Barr virus, malaria, Sjogren syndrome, and lupus.
In order to reduce the chances of developing MZL, an individual can decrease their exposure to the possible risk factors.
Of all cancers involving the same class of blood cell, 2.3% of cases are Burkitt lymphoma. Epstein-Barr virus infection is strongly correlated with this cancer.
The Center for Disease Control and Prevention (CDC) included certain types of non-Hodgkin's lymphoma as AIDS-defining cancers in 1987. Immune suppression rather than HIV itself is implicated in the pathogenesis of this malignancy, with a clear correlation between the degree of immune suppression and the risk of developing NHL. Additionally, other retroviruses such as HTLV may be spread by the same mechanisms that spread HIV, leading to an increased rate of co-infection. The natural history of HIV infection has been greatly changed over time. As a consequence, rates of non-Hodgkin's lymphoma (NHL) in people infected with HIV has significantly declined in recent years.
Drinking alcohol excessively is a major cause of chronic pancreatitis, which in turn predisposes to pancreatic cancer. However, considerable research has failed to firmly establish alcohol consumption as a direct risk factor for pancreatic cancer. Overall, the association is consistently weak and the majority of studies have found no association, with smoking a strong confounding factor. The evidence is stronger for a link with heavy drinking, of at least six drinks per day.
The most common chemotherapy used for non-Hodgkin lymphoma is R-CHOP.
Treatment of Hodgkin's disease has been improving over the past few decades. Recent trials that have made use of new types of chemotherapy have indicated higher survival rates than have previously been seen. In one recent European trial, the 5-year survival rate for those patients with a favorable prognosis (FFP) was 98%, while that for patients with worse outlooks was at least 85%.
In 1998, an international effort identified seven prognostic factors that accurately predict the success rate of conventional treatment in patients with locally extensive or advanced stage Hodgkin's lymphoma. Freedom from progression (FFP) at 5 years was directly related to the number of factors present in a patient. The 5-year FFP for patients with zero factors is 84%. Each additional factor lowers the 5-year FFP rate by 7%, such that the 5-year FFP for a patient with 5 or more factors is 42%.
The adverse prognostic factors identified in the international study are:
- Age ≥ 45 years
- Stage IV disease
- Hemoglobin < 10.5 g/dl
- Lymphocyte count < 600/µl or < 8%
- Male
- Albumin < 4.0 g/dl
- White blood count ≥ 15,000/µl
Other studies have reported the following to be the most important adverse prognostic factors: mixed-cellularity or lymphocyte-depleted histologies, male sex, large number of involved nodal sites, advanced stage, age of 40 years or more, the presence of B symptoms, high erythrocyte sedimentation rate, and bulky disease (widening of the mediastinum by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension.)
More recently, use of positron emission tomography (PET) early after commencing chemotherapy has demonstrated to have powerful prognostic ability. This enables assessment of an individual's response to chemotherapy as the PET activity switches off rapidly in patients who are responding. In this study, after two cycles of ABVD chemotherapy, 83% of patients were free of disease at 3 years if they had a negative PET versus only 28% in those with positive PET scans. This prognostic power exceeds conventional factors discussed above. Several trials are underway to see if PET-based risk adapted response can be used to improve patient outcomes by changing chemotherapy early in patients who are not responding.
Chemotherapy is the mainstay of treatment for lymphoma in cats. Most of the drugs used in dogs are used in cats, but the most common protocol uses cyclophosphamide, vincristine, and prednisone. Gastrointestinal lymphoma has also commonly been treated with a combination of prednisolone and high dose pulse chlorambucil with success. The white blood cell count must be monitored. Remission and survival times are comparable to dogs. Lower stage lymphoma has a better prognosis. Multicentric lymphoma has a better response to treatment than the gastrointestinal form, but infection with FeLV worsens the prognosis.
About 75% of cats treated with chemotherapy for lymphoma go into remission. Unfortunately, after an initial remission, most cats experience a relapse, after which they have a median survival of 6 months. However, about one-third of cats treated with chemotherapy will survive more than 2 years after diagnosis; a small number of these cats may be cured of their disease. Untreated, most cats with lymphoma die within 4–6 weeks. Most cats tolerate their chemotherapy well, and fewer than 5% have severe side effects. Cats do not lose their fur from chemotherapy, though loss of whiskers is possible. Other side effects include low white blood cell count, vomiting, loss of appetite, diarrhea, or fatigue. These can typically be controlled well, and most cats have a good quality of life during treatment. If a cat relapses after attaining remission, the cat can be treated with different chemotherapy drugs to try for a second remission. The chances of a second remission are much lower than the chances of obtaining a first, and the second remission is often shorter than the first.
Lymphoma is the most common form of hematological malignancy, or "blood cancer", in the developed world.
Taken together, lymphomas represent 5.3% of all cancers (excluding simple basal cell and squamous cell skin cancers) in the United States and 55.6% of all blood cancers.
According to the U.S. National Institutes of Health, lymphomas account for about 5%, and Hodgkin lymphoma in particular accounts for less than 1% of all cases of cancer in the United States.
Because the whole system is part of the body's immune system, patients with a weakened immune system such as from HIV infection or from certain drugs or medication also have a higher incidence of lymphoma.
Median survival is around 10 years, but the range is wide, from less than one year, to more than 20 years. Some patients may never need treatment. The overall survival rate at five years is 72–77%. Recent advances and addition of Rituximab, improved median survival. Recent reports for the period 1986 and 2012 estimates median survival of over 20 years.
Unlike some other lymphomas, whose incidence increases with age, Hodgkin's lymphoma has a bimodal incidence curve; that is, it occurs most frequently in two separate age groups, the first being young adulthood (age 15–35) and the second being in those over 55 years old although these peaks may vary slightly with nationality. Overall, it is more common in males, except for the nodular sclerosis variant, which is slightly more common in females. The annual incidence of Hodgkin's lymphoma is 2.7 per 100,000 per persons per year, and the disease accounts for slightly less than 1% of all cancers worldwide.
In 2010, globally it resulted in about 18,000 deaths down from 19,000 in 1990.
The incidence of Hodgkin's lymphoma is increased in patients with HIV infection. In contrast to many other lymphomas associated with HIV infection it occurs most commonly in patients with higher CD4 T cell counts.
Orbital lymphoma accounts for 55% of malignant orbital tumors in adults. In one study, this was 10% of patients presenting with orbital tumors or similar lesions. Orbital lymphoma is more prevalent in Asia and Europe than in the United States.
Although intraocular lymphoma is rare, the number of cases per year is rising, affecting mainly people in their seventies and immunocompromised patients. A recent study has shown that ocular lymphoma is more prevalent in women than men.
The survival rate is approximately 60% after 5 years.
Untreated dogs have an average survival time of 60 days. Lymphoma with a histologic high grade generally respond better to treatment but have shorter survival times than dogs with low grade lymphoma. Dogs with B-lymphocyte tumors have a longer survival time than T-lymphocyte tumors. Mediastinal lymphoma has a poorer prognosis than other types, especially those with hypercalcemia. Clinical stage and substage have some prognostic value, with poorer prognosis associated with Stage V disease, and with substage b (clinical illness at time of presentation).
An individuals prognosis can be based on the Ann Arbor Staging System. If an individual is diagnosed with Stage I marginal zone B-cell NHL, this indicates that there is involvement of a single lymph node region and/or involvement of a single extra-lymphatic organ. Stage II indicates that two or more lymph node regions on the same side of the diaphragm and/or involvement of an extra-lymphatic organ or site and one or more lymph node on the same side of the diaphragm. Stage III indicates involvement of lymph nodes on both sides of the diaphragm, splenic involvement, and extra lymphatic site involvement. Stage IV indicates involvement of more than one extra lymphatic organ or tissues without lymph node involvement. If symptoms such as fever, night sweats, and more than a 10% weight loss are experienced within the first six months, this is characterized by a "B" for present. If these symptoms are absent this is characterized by an "A" for abscent. Extra-nodal sites are characterized as follows: marrow (M+), lungs (L+), liver (H+), pleura (P+), bone (O+), and skin and subcutaneous tissue (D+).
Nodal MZL not only develops at a very slow rate but often relapses in individuals. These individuals often survive for a long time with treatment at various points in their life to keep the cancer at bay. MALT lymphoma also develops at a slow rate and is usually treated successfully even when present in various areas of the body. Splenic MZL also develops at a slow rate but is usually incurable. Even though it is not generally cured, it can be managed for many years
It is important to note that the prognosis of individual cases varies and further information will be available through a physician.
Due to the causal relationship between "H. pylori" infection and MALT lymphoma, identification of the infection is mandatory. Histological examination of GI biopsies yields a sensitivity of 95% with five biopsies, but these should be from sites uninvolved by lymphoma and the identification of the organism may be compromised by areas of extensive intestinal metaplasia. As proton-pump inhibition can suppress infection, any treatment with this class of drug should be ceased 2 weeks prior to biopsy retrieval. Serology should be performed if histology is negative, to detect suppressed or recently treated infections. Following the recognition of the association of gastric MALT lymphoma with "H. pylori" infection, it was established that early-stage gastric disease could be cured by "H. pylori" eradication, which is now the mainstay of therapy. Fifty to 95% of cases achieve complete response (CR) with "H. pylori" treatment.
A t(11;18)(q21;q21) chromosomal translocation, giving rise to an "API2-MLT" fusion gene, is predictive of poor response to eradication therapy.
Thyroid lymphoma is a rare malignant tumor constituting 1% to 2% of all thyroid malignancies and less than 2% of lymphomas. Thyroid lymphomas are classified as non–Hodgkin's B-cell lymphomas in a majority of cases, although Hodgkin's lymphoma of the thyroid has also been identified.
Primary cerebral lymphoma (or "primary central nervous system lymphoma") is a form of NHL. It is very rare in immunocompetent people, with an incidence of 5–30 cases per million person-years. However the incidence in immunocompromised individuals is greatly increased, up to 100 per million person-years.
Primary cerebral lymphoma is strongly associated with Epstein–Barr virus (EBV). The presence of EBV DNA in cerebrospinal fluid is highly suggestive of primary cerebral lymphoma.
Treatment of AIDS patients with antiretroviral drugs reduces the incidence of primary cerebral lymphoma.
The incidence of Hodgkin's disease in the general population is about 10–30 per million person-years. This increases to 170 per million person-years in HIV positive patients.
Treatment with dose-adjusted EPOCH with rituximab has shown promising initial results in a small series of patients (n=17), with a 100% response rate, and 100% overall survival and progression-free survival at 28 months (median follow-up).
Palliative care, a specialized medical care focused on the symptoms, pain, and stress of a serious illness, is recommended by multiple national cancer treatment guidelines as an accompaniment to curative treatments for people suffering from lymphoma. It is used to address both the direct symptoms of lymphoma and many unwanted side effects that arise from treatments. Palliative care can be especially helpful for children who develop lymphoma, helping both children and their families deal with the physical and emotional symptoms of the disease. For these reasons, palliative care is especially important for patients requiring bone marrow transplants.