Indolent lymphoma

Patients normally present with painless, swollen lymph nodes, often in the neck, armpit, or groin. Some have swollen lymph nodes inside their body, such as in the chest or abdomen, which can go undetected until they become very large and cause symptoms like cough or abdominal pain. == Risk factors ==

The cause of indolent lymphoma is unknown, but research has identified a number of factors that increase risk. Age The risk of developing indolent lymphoma increases with age. Although the disease can occur at any age, it mainly affects older adults. It is uncommon in people under age 40 and very rare in children. Infection People with HIV are at slightly higher risk than the general population. For patients who were previously diagnosed with an AIDS-defining illness, the risk of developing indolent lymphoma is 14-fold higher. Other factors First-degree family history of non-Hodgkin lymphoma, hematological malignancy, or hepatitis C infection are linked to an increased risk of indolent lymphoma. There are also specific risk factors for individual subtypes. Higher body mass index (BMI) as a young adult, occupation as a spray painter, sedentary lifestyle, and high levels of dietary animal protein intake are associated with higher risk of follicular lymphoma. Living on a farm is associated with an increased risk of mantle cell lymphoma. Sjögren's syndrome, systemic lupus erythematosus, and tobacco smoking for more than 40 years are linked to an increased risk of Waldenstrom macroglobulinemia. == Diagnosis ==

Classification With a few exceptions, indolent lymphomas are of B cell origin. They are classified based on pathological and cytological features. Follicular lymphoma Follicular lymphoma (FL) is a lymphoproliferative disorder generally associated with an indolent course. It originates from follicular center B cells. It is most frequently diagnosed among people in their 50s and 60s, and is more common among white populations than black or Asian populations. ==== Cutaneous T-cell lymphoma ==== Unlike most NHL subtypes, cutaneous T-cell lymphoma (CTCL) is derived from T cells. Mycosis fungoides, which attacks the skin, is the most common form of CTCL. When cancer cells infiltrate and accumulate in the blood, it is known as Sézary syndrome. The median age at diagnosis is 67 years, and the disease is slightly more common in women than in men. The World Health Organization categorizes MZL into three subtypes: nodal, extranodal, and splenic. Chronic lymphocytic leukemia and small cell lymphocytic lymphoma Chronic lymphocytic leukemia (CLL) and small cell lymphocytic lymphoma (SLL) are different manifestations of the same disease and are managed in the same way. When the abnormal lymphocytes are located mostly in the lymph nodes, it is referred to as SLL; when the abnormal lymphocytes are mostly in the blood and bone marrow, it is called CLL. CLL is the most common leukemia in Western countries, but it is very rare in East Asia. The median age at diagnosis is 72 years. Stage I: The lymphoma is in one lymph node or one group of lymph nodes; or, in rare cases, in one organ of the lymphatic system such as Waldeyer's ring, the thymus, or the spleen; or in one site outside the lymphatic system (IE). Stage II: The lymphoma is in two or more groups of lymph nodes; or in one nearby area outside the lymphatic system, with or without involvement of other lymph nodes (IIE). In either case, the lymphoma sites are on the same side of the diaphragm. In Stage II, "bulky disease" means tumor mass larger than a certain size; the threshold depends on the type of lymphoma. Stage III: The lymphoma is on both sides of the diaphragm, either in lymph nodes both above and below the diaphragm, or in lymph nodes above the diaphragm and in the spleen. Stage IV: The lymphoma is in one or more organs beyond the lymphatic system, such as the liver, lungs, bone marrow, or cerebrospinal fluid. A patient's stage may be determined through blood tests, bone marrow biopsy, chest X-rays, computed tomography (CT) scans, positron emission tomography (PET) scans, and magnetic resonance imaging (MRI). == Treatment ==

Indolent lymphoma tends to grow slowly. As a result, patients may not need to start treatment immediately upon diagnosis. Instead, they may be closely monitored—an approach known as watchful waiting—and start treatment when the disease progresses and causes symptoms, Patients with early-stage indolent lymphoma may be cured with radiation therapy, but most patients have widespread disease at the time of diagnosis. There are many effective treatments to control later stages, but they are not reliably curative. Allogeneic stem cell transplantation can be curative because of the potential for immunologic graft-versus-lymphoma effect, but there are significant concerns regarding non-relapse mortality. Treatment by stage Stage I and contiguous Stage II External radiation therapy (or external beam radiation therapy) may be given to affected lymph nodes and nearby lymph nodes. Other treatments include chemotherapy and monoclonal antibody therapy with rituximab. Clinical trials are also an option. Marginal zone lymphoma Gastric MZL is often related to Helicobacter pylori infection. Many patients can be cured with antibiotics alone. If remission is not achieved, radiation therapy may be used. For nodal MZL that involves the spleen and blood, treatment is similar to that of follicular lymphoma. == Prognosis ==

Although indolent lymphoma tends to progress slowly and median overall survival is more than 10 years, prognoses differs substantially both within and between subtypes. Some patients live many years longer than the median survival, while others die a short time after diagnosis. Patients with HIV infection tend to have similar median survival as patients who are HIV negative. A study in the Netherlands shows that in the younger age group, mortality caused by follicular lymphoma and marginal zone lymphoma after 15 years is minimal, suggesting the likelihood of a cure. == References ==