<unk>  is a subtype of B-cell lymphoma characterized by the presence of CD5 positive antigen-naive, pregerminal center, B-cells within the<unk>  zone that surrounds normal germinal center follicles.<unk>  accounts for 4–8% of all adult non-Hodgkin’s lymphomas (NHL). It has an incidence of approximately 1-2/100000 and tends to occur more in males, Caucasian race, with a median age of about 60 years.The translocation t(11;14)(q13;q32) is considered the precipitating oncogenic event that induces cell cycle deregulation in<unk>  due to overexpression of cyclin D1. In addition to the pathogonomic translocation,<unk>  progression is controlled by secondary genetic abberations and dysregulated signaling pathways involved in DNA damage repair, proliferation, and apoptosis. According to the revised 2016 World Health Organization classification of lymphoid neoplasms,<unk>  <unk>  can be broadly classified into classical<unk>  and leukemic nonnodal<unk> . In-situ<unk>  cell neoplasia (ISMCN) is considered a separate entity, often as a precursor lesion, to the<unk> . The causes of<unk>  have not been clearly identified. There are no established risk factors for<unk> . However, recently weak associations have been observed in the development of<unk>  with the exposure to European strains of Borellia burgdoferi, family history of hematologic malignancy and Genetic polymorphisms in the pro-inflammatory cytokine IL-10.<unk>  must be differentiated from other diseases that present similarly with B symptoms (fever, night sweats and unexplained weight loss), lymphocytosis, lymphadenopathy, hepatosplenomegaly, and bone marrow involvement.<unk>  must be differentiated from other diseases such as diffuse large B cell lymphoma, mucosa-associated lymphatic tissue lymphoma (MALT), follicular lymphoma, small lymphocytic lymphoma/chronic lymphocytic leukemia, lymphoplasmacytoid lymphoma/Immunocytoma, marginal zone lymphoma, lymphoblastic lymphoma, burkitt lymphoma and reactive hyperplasia. Screening for<unk>  is not recommended. The prognosis of<unk>  has historically been very poor. However, recently improvements have been made and the median survival has increased from 3-4 years to 5-7 years. It is very important to stratify the patients according to their biological risk to better direct the therapeutic approaches. Tissue biopsy (nodal or extranodal) is the gold standard test for the diagnosis of<unk> . The most common symptoms of<unk>  include fever, weight loss, night sweats, fatigue, skin rash, abdominal pain, bone pain, loss of appetite, and painless swelling in the neck, axilla, groin, thorax and abdomen. Common physical examination findings of<unk>  include fever, rash, splenomegaly, hepatomegaly, peripheral lymphadenopathy, and central lymphadenopathy. Laboratory tests for<unk>  include complete blood count (CBC), comprehensive metabolic panel, LDH levels, Hepatitis B testing if treatment with rituximab is planned, uric acid levels, beta-2-microglobulin, pregnancy testing in woman of child-bearing age, flow cytometry, immunohistochemistry, genetic testing and FISH. Ultrasound may be helpful in the diagnosis of<unk> . Findings on an ultrasound helpful in the diagnosis of<unk>  include lymphadenopathy, splenomegaly and hepatomegaly. There are no echocardiography findings associated with<unk> . However, an echocardiography may be helpful in following patients who might be at risk of anthracycline-induced cardiotoxicity due to chemotherapy. CT scan may be helpful in the diagnosis of<unk> . CT scans of the chest, abdominal and pelvic are done to check for lymphadenopathy and is helpful in the staging the disease. MRI may be helpful in the diagnosis of<unk>  involving the CNS. PET scan (positron emission tomography) integrated with CT scan may be helpful in the staging and treatment response assessment of<unk> . Other diagnostic studies for the diagnosis of<unk>  include bone marrow aspiration, lumbar puncture, colonoscopy, upper endoscopy, laparoscopy, and laparotomy.The mainstay of treatment for<unk>  is chemotherapy. However, immunotherapy, radioimmunotherapy, targeted therapy using newer biologic agents and stem cell transplantation are also used along with chemotherapy to treat the disease.<unk>  shows a heterogeneous clinical behavior, with some patients having indolent disease whereas a vast majority show aggressive presentation. Most of the patients eventually relapse and have disease progression after treatment. Hence,<unk>  is still considered an incurable disease and there is no consensus among oncologists about its optimal treatment. It is therefore recommended that<unk>  patients are seen by physicians having extensive experience in dealing with<unk>  and they are also encouraged to participate in clinical trials to get the latest treatments. Recent advances in the understanding of the pathogenesis of<unk>  have led to the development of targeted therapies which have shown potential promise as effective therapeutic approaches in the future.
Mantle cell lymphoma
Anaplastic large cell lymphoma
Diffuse large B cell lymphoma
Follicular lymphoma
Nodal marginal zone lymphoma
AIDS-related lymphoma
T-cell lymphoma
B-cell lymphoma