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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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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.
Of all cancers involving the same class of blood cell, 8% of cases are MALT 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.
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.
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.
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.
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.
As other thyroid lesions, thyroid lymphoma affects predominantly females over 70 years of age with a history of Hashimoto's thyroiditis. Thus, Hashimoto's thyroiditis is considering as risk factor for thyroid lymphoma development.
The thyroid lymphoma manifests as rapidly enlarging neck mass causing respiratory difficulty. On physical examination, patients usually exhibit a firm thyroid and lymphadenopathy.
This rare form of leukemia is more common among Asians in comparison to other ethnic groups. It is typically diagnosed in adolescents and young adults, with a slight predominance in males.
Additionally, some researchers separate out lymphomas that appear to result from other immune system disorders, such as AIDS-related lymphoma.
Classic Hodgkin's lymphoma and nodular lymphocyte predominant Hodgkin's lymphoma are now considered forms of B-cell lymphoma.
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.
The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph nodes. They develop more frequently in older adults and in immunocompromised individuals.
B-cell lymphomas include both Hodgkin's lymphomas and most non-Hodgkin lymphomas. They are typically divided into low and high grade, typically corresponding to indolent (slow-growing) lymphomas and aggressive lymphomas, respectively. As a generalisation, indolent lymphomas respond to treatment and are kept under control (in remission) with long-term survival of many years, but are not cured. Aggressive lymphomas usually require intensive treatments, with some having a good prospect for a permanent cure.
Prognosis and treatment depends on the specific type of lymphoma as well as the stage and grade. Treatment includes radiation and chemotherapy. Early-stage indolent B-cell lymphomas can often be treated with radiation alone, with long-term non-recurrence. Early-stage aggressive disease is treated with chemotherapy and often radiation, with a 70-90% cure rate. Late-stage indolent lymphomas are sometimes left untreated and monitored until they progress. Late-stage aggressive disease is treated with chemotherapy, with cure rates of over 70%.
"MALT lymphoma" is exquisitely immunotherapy sensitive. Chemotherapy is reserved for those uncommon patients with disseminated disease at presentation or lack of response to local treatment. Rituximab, the anti-CD20 chimeric antibody, is a key component of therapy. Responses vary from 55% to 77% with monotherapy and 100% in combination with chemotherapy. Oral alkylating agents such as cyclophosphamide or chlorambucil have been administered for a median duration of 12 months with high rates of disease control (CR up to 75%) but appear not to be active in t(11;18) disease. The purine nucleoside analogs fludarabine and cladribine also demonstrate activity, the latter conferring a CR rate of 84% (100% in those with gastric primaries) in a small study. A pivotal study of rituximab plus chlorambucil compared with chlorambucil alone (IELSG-19 study, n = 227) demonstrated a significantly higher CR rate (78% vs. 65%; p = 0.017) and 5-year EFS (68% vs. 50%; p = 0.024) over chlorambucil alone. However, 5-year OS was not improved (88% in both arms). First-line treatment of choice is generally rituximab in combination with single alkylating agents or fludarabine, or a combination of all three drugs. The final results of this study, including the later addition of a rituximab-alone arm, are pending.
Two other genetic alterations are known:
- t(1;14)(p22;q32), which deregulates BCL10, at the locus 1p22.
- t(14;18)(q32;q21), which deregulates MALT1, at the locus 18q21.
These seem to turn on the same pathway as API2-MLT (i.e., that of NF-κB). They both act upon IGH, which is at the locus 14q32.
Pralatrexate is one compound currently under investigations for the treatment of PTCL.
Cutaneous lymphoma, also known as lymphoma cutis, is when lymphoma involves the skin. It is characterized by a proliferation of lymphoid tissue.
There are two main classes of lymphomas that affect the skin:
- Cutaneous T-cell lymphoma
- Cutaneous B-cell lymphoma
Taken together, haematological malignancies account for 9.5% of new cancer diagnoses in the United States and 30,000 patients in the UK are diagnosed each year. Within this category, lymphomas are more common than leukemias.
T-cell leukemia describes several different types of lymphoid leukemia which affect T cells.
Types include:
- Large granular lymphocytic leukemia
- Adult T-cell leukemia/lymphoma
- T-cell prolymphocytic leukemia
In practice, it can be hard to distinguish T-cell leukemia from T-cell lymphoma, and they are often grouped together.
Lymphoid leukemias — also called lymphocytic, lymphogenous, or lymphoblastic leukemias — are a group of leukemias affecting circulating lymphocytes, a type of white blood cells. The lymphocytic leukemias are closely related to lymphomas of the lymphocytes, to the point that some of them are unitary disease entities that can be called by either name (for example, adult T-cell leukemia/lymphoma). Such diseases are all lymphoproliferative disorders. Most lymphoid leukemias involve a particular subtype of lymphocytes, the B cells.
Historically, they have been most commonly divided by the stage of maturation at which the clonal (neoplastic) lymphoid population stopped maturing:
- Acute lymphoblastic leukemia
- Chronic lymphocytic leukemia
However, the influential WHO Classification (published in 2001) emphasized a greater emphasis on cell lineage. To this end, lymphoid leukemias can also be divided by the type of cells affected:
- B-cell leukemia
- T-cell leukemia
- NK-cell leukemia
The most common type of lymphoid leukemia is B-cell chronic lymphocytic leukemia.
Historically, hematological malignancies have been most commonly divided by whether the malignancy is mainly located in the blood (leukemia) or in lymph nodes (lymphomas).
However, the influential WHO Classification (published in 2001) placed a greater emphasis on cell lineage.
Relative proportions of hematological malignancies in the United States
Some specific reactive lymphadenopathies with a predominantly follicular pattern:
- Rheumatoid arthritis
- Sjogren syndrome
- IgG4-related disease (IgG4-related lymphadenopathy)
- Kimura disease
- Toxoplasmosis
- Syphilis
- Castleman disease
- HIV-associated lymphadenopathy
- Progressive transformation of germinal centers (PTGC)
Follicular hyperplasia (or "reactive follicular hyperplasia" or "lymphoid nodular hyperplasia") is a type of lymphoid hyperplasia. It is caused by a stimulation of the B cell compartment. It is caused by an abnormal proliferation of secondary follicles and occurs principally in the cortex without broaching the lymph node capsule. The follicles are cytologically polymorphous, are often polarized, and vary in size and shape. Follicular hyperplasia is distinguished from follicular lymphoma in its polyclonality and lack of bcl-2 protein expression, whereas follicular lymphoma is monoclonal, and does express bcl-2).
Treatment is as with hypothyroidism, daily thyroxine(T4) and/or triiodothyronine(T3).
Outside Europe a goitrous form of autoimmune thyroiditis (Hashimoto's Thyroiditis) is more common than Ord's disease.
Lymphocyte-variant hypereosinophilia usually takes a benign and indolent course. Long term treatment with corticosteroids lowers blood eosinophil levels as well as suppresses and prevents complications of the disease in >80% of cases. However, signs and symptoms of the disease recur in virtually all cases if corticosteroid dosages are tapered in order to reduce the many adverse side effects of corticosteroids. Alternate treatments used to treat corticosteroid resistant disease or for use as corticosteroid-sparing substitutes include interferon-α or its analog, Peginterferon alfa-2a, Mepolizumab (an antibody directed against IL-5), Ciclosporin (an Immunosuppressive drug), imatinib (an inhibitor of tyrosine kinases; numerous tyrosine kinase cell signaling proteins are responsible for the growth and proliferation of eosinophils {see clonal eosinophilia}), methotrexate and Hydroxycarbamide (both are chemotherapy and immunosuppressant drugs), and Alemtuzumab (a antibody that binds to the CD52 antigen on mature lymphocytes thereby marking them for destruction by the body). The few patients who have been treated with these alternate drugs have exhibited good responses in the majority of instances. Reslizumab, a newly developed antibody directed against interleukin 5 that has been successfully used to treat 4 patients with the hypereosinophilic syndrome, may also be of use for lymphocyte-variant eosinophilia. Patients suffering minimal or no disease complications have gone untreated.
In 10% to 25% of patients, mostly 3 to 10 years after initical diagnosis, the indolent course of lymphocyte-variant hypereosinophilia changes. Patients exhibit rapid increases in lymphadenopathy, spleen size, and blood cell numbers, some cells of which take on the appearance of immature and/or malignant cells. Their disease soon thereafter escalates to an angioimmunoblastic T-cell lymphoma, peripheral T cell lymphoma, Anaplastic large-cell lymphoma (which unlike most lymphomas of this type is Anaplastic lymphoma kinase-negative), or Cutaneous T cell lymphoma. The malignantly transformed disease is aggressive and has a poor prognosis. Recommended treatment includes chemotherapy with Fludarabine, Cladribine, or the CHOP combination of drugs followed by bone marrow transplantation.