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Most individuals with non-gastric MALT have no symptoms
- Symptoms depend on where the cancer originates:
- Mass in the salivary gland
- Redness and sensitivity of the eye
- Mass in the thyroid
- Problems swallowing
- Cough
- Shortness of breath
- Fever
- Weight loss
- Red-brown discoloration of the skin
Individuals with this type of cancer experience almost no symptoms at all.
- Painless Lymphadenopathy
- Fatigue
- Weight loss
- Fevers
- Night sweat
The presentation depends if it is mycosis fungoides or Sézary syndrome the most common, though not the only types.
Among the symptoms for the aforementioned types are:lymphadenopathy, hepatosplenomegaly, and non-specific dermatitis.
The clinical presentation varies according to the type of ALCL. Two of the ALCL subtypes are systemic lymphomas, in that they usually present with enlarged lymph nodes in multiple regions of the body, or with tumors outside the lymph nodes (extranodal) such as bone, intestine, muscle, liver, or spleen. These 2 subtypes usually associate with weight loss, fevers and night sweats, and can be lethal if left untreated without chemotherapy. The third type of ALCL is so-called cutaneous ALCL, and is a tumor that presents in the skin as ulcers that may persist, or occasionally may involute spontaneously, and commonly recur. This type of ALCL usually manifests in different regions of the body and may extend to regional lymph nodes, i.e., an axillary lymph node if the ALCL presents in the arm.
A rare subtype of ALCL has been identified in a few women who have silicone breast implants (protheses) as a result of breast reconstruction after a diagnosis of breast cancer. The tumor initially manifests with swelling of the breast due to fluid accumulation around the implant. The disease may progress to invade the tissue surrounding the capsule, and if left untreated may progress to the axillary lymph nodes.
It typically presents at a late stage and is often associated with systemic symptoms ("B symptoms").
The symptoms of AIDS-related lymphoma can include: weight loss, fever, and night sweats.
Lymphoma may present with certain nonspecific symptoms; if the symptoms are persistent, an evaluation to determine their cause, including possible lymphoma, should be undertaken.
- Lymphadenopathy or swelling of lymph nodes, is the primary presentation in lymphoma.
- B symptoms (systemic symptoms) – can be associated with both Hodgkin lymphoma and non-Hodgkin lymphoma. They consist of:
- Fever
- Night sweats
- Weight loss
- Other symptoms:
- Loss of appetite or anorexia
- Fatigue
- Respiratory distress or dyspnea
- Itching
A point-based algorithm for the diagnosis for early forms of cutaneous T cell lymphoma was proposed by The International Society for Cutaneous Lymphomas in 2005.
Extranodal NK/T-cell lymphoma, nasal type which was known as angiocentric lymphoma in the REAL classification, and also as nasal-type NK lymphoma, NK/T-cell lymphoma, and polymorphic/malignant midline reticulosis is a cutaneous condition which in Korea is reported to be the most common form of cutaneous lymphoma after mycosis fungoides.
Extranodal NK-T-cell lymphoma is a type of lymphoma.
It is called "extranodal" to emphasize that the location is typically not in the lymph node, and is sometimes further qualified as "nasal type". The nasal cavity, nasopharynx and upper aerodigestive tract are often involved, although extranasal presentations do occur (skin, gastrointestinal tract, eye, testis, lung, soft tissue). There is a strong association with Epstein–Barr virus.
The NCCN guidelines recommend either high-dose radiotherapy alone for stage I without high risk features, or concurrent chemoradiotherapy for stage I and II with either of two regimens. Asparaginase containing regimens have been used in advanced stage disease.
The signs and symptoms of non-Hodgkin's lymphoma vary depending upon its location within the body. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and feeling tired. Other symptoms may include bone pain, chest pain, or itchiness. Some forms are slow growing while others are fast growing. Enlarged lymph nodes may cause lumps to be felt under the skin when they are close to the surface of the body. Lymphomas in the skin may also result in lumps, which are commonly itchy, red or purple. Lymphomas in the brain can cause weakness, seizures, problems with thinking and personality changes.
ATL is usually a highly aggressive non-Hodgkin's lymphoma with no characteristic histologic appearance except for a diffuse pattern and a mature T-cell phenotype. Circulating lymphocytes with an irregular nuclear contour (leukemic cells) are frequently seen. Several lines of evidence suggest that HTLV-1 causes ATL. This evidence includes the frequent isolation of HTLV-1 from patients with this disease and the detection of HTLV-1 proviral genome in ATL leukemic cells. ATL is frequently accompanied by visceral involvement, hypercalcemia, skin lesions, and lytic bone lesions. Bone invasion and osteolysis, features of bone metastases, commonly occur in the setting of advanced solid tumors, such as breast, prostate, and lung cancers, but are less common in hematologic malignancies. However, patients with HTLV-1–induced ATL and multiple myeloma are predisposed to the development of tumor-induced osteolysis and hypercalcemia. One of the striking features of ATL and multiple myeloma induced bone disease is that the bone lesions are predominantly osteolytic with little associated osteoblastic activity. In patients with ATL, elevated serum levels of IL-1, TGFβ, PTHrP, macrophage inflammatory protein (MIP-1α), and receptor activator of nuclear factor-κB ligand (RANKL) have been associated with hypercalcemia. Immunodeficient mice that received implants with leukemic cells from patients with ATL or with HTLV-1–infected lymphocytes developed hypercalcemia and elevated serum levels of PTHrP. Most patients die within one year of diagnosis.
Infection with HTLV-1, like infection with other retroviruses, probably occurs for life and can be inferred when antibody against HTLV-1 is detected in the serum.
Currently, Burkitt lymphoma can be divided into three main clinical variants: the endemic, the sporadic, and the immunodeficiency-associated variants.
- The endemic variant (also called "African variant") most commonly occurs in children living in malaria endemic regions of the world (e.g., equatorial Africa, Brazil, and Papua New Guinea). Epstein-Barr virus (EBV) infection is found in nearly all patients. Chronic malaria is believed to reduce resistance to EBV, allowing it to take hold. The disease characteristically involves the jaw or other facial bone, distal ileum, cecum, ovaries, kidney, or breast.
- The sporadic type of Burkitt lymphoma (also known as "non-African") is the most common variant found in places where malaria is not holoendemic. The tumor cells have a similar appearance to the cancer cells of classical endemic Burkitt lymphoma. Sporadic lymphomas are rarely associated with the Epstein–Barr virus. Non-Hodgkin lymphoma, which includes Burkitt's, accounts for 30–50% of childhood lymphoma. The jaw is less commonly involved, compared to the endemic variant. The ileocecal region is the common site of involvement.
- Immunodeficiency-associated Burkitt lymphoma is usually associated with HIV infection or occurs in the setting of post-transplant patients who are taking immunosuppressive drugs. Burkitt lymphoma can be one of the diseases associated with the initial manifestation of AIDS.
By morphology (i.e., microscopic appearance) or immunophenotype, it is almost impossible to differentiate these three clinical variants. Immunodeficiency-associated Burkitt lymphoma may demonstrate more plasmacytic appearance or more pleomorphism, but these features are not specific.
The median age of diagnosis is approximately fourth and fifth decades. The disease often arises in the oral cavity, particularly in HIV disease, but other sites include nasal cavity, gastrointestinal tract, skin, bone soft tissue, and lung. There is usually immunodeficiency, such as HIV, organ transplants, autoimmune diseases
Like Burkitt's lymphoma, the morphology has a "starry sky". However, the immunophenotype resembles plasma cells: CD45-, CD20-, CD79a+/-, PAX5-, CD38+, CD38+ and MUM1+. Ki67 is over 90%. EBV is positive in 75%; HHV-8 is negative.
The typical clinical finding in a patient with hepatosplenic T-cell lymphoma is hepatosplenomegaly.
Peripheral T-cell lymphoma refers to a group of T-cell lymphomas that develop away from the thymus.
Examples include:
- Cutaneous T-cell lymphomas
- Angioimmunoblastic T-cell lymphoma
- Extranodal natural killer/T-cell lymphoma, nasal type
- Enteropathy type T-cell lymphoma
- Subcutaneous panniculitis-like T-cell lymphoma
- Anaplastic large cell lymphoma
- Peripheral T-cell lymphoma-Not-Otherwise-Specified
In ICD-10, cutaneous T-cell lymphomas are classified separately.
The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.
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
At diagnosis, patients typically are in their 60s and present to their physician with advanced disease. About half have either fever, night sweats, or unexplained weight loss (over 10% of body weight). Enlarged lymph nodes (for example, a "bump" on the neck, armpits or groin) or splenomegaly are usually present. Bone marrow, liver and GI tract involvement occurs relatively early in the course of the disease.
Diffuse large B-cell lymphoma encompasses a biologically and clinically diverse set of diseases, many of which cannot be separated from one another by well-defined and widely accepted criteria. The World Health Organization (WHO) classification system defines more than a dozen subtypes, each of which can be differentiated based on the location of the tumor, the presence of other cells within the tumor (such as T cells), and whether the patient has certain other illnesses related to DLBCL. One of these well-defined groupings of particular note is "primary mediastinal (thymic) large B cell lymphoma", which arises within the thymus or mediastinal lymph nodes.
In some cases, a tumor may share many features with both DLBCL and Burkitt's lymphoma. In these situations, the tumor is classified as simply “B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma”. A similar situation can arise between DLBCL and Hodgkin's lymphoma; the tumor is then classified as “B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Hodgkin’s lymphoma”.
When a case of DLBCL does not conform to any of these subtypes, and is also not considered unclassifiable, then it is classified as “diffuse large B-cell lymphoma, not otherwise specified” (DLBCL, NOS). The majority of DLBCL cases fall into this category. Much research has been devoted to separating this still-heterogeneous group; such distinctions are usually made along lines of cellular morphology, gene expression, and immunohistochemical properties.
Burkitt lymphoma is a cancer of the lymphatic system, particularly B lymphocytes found in the germinal center. It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1958 while working in equatorial Africa.
Patients with this disease usually present at an advanced stage and show systemic involvement. The clinical findings typically include a pruritic skin rash and possibly edema, ascites, pleural effusions, and arthritis.
The T-cell lymphomas are four types of lymphoma that affect T cells. These account for about one in ten cases of non-Hodgkin lymphoma.
They can be associated with Epstein Barr virus and Human T-cell leukemia virus-1.
One classification system for lymphomas divides the diseases according to the size of the white blood cells that has turned cancerous. The large-cell lymphomas have large cells. A large cell, in this context, has a diameter of 17 to 20 µm. Other groups of lymphomas in this system are the small-cell lymphomas and mixed-cell lymphomas.
Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), is a subtype of peripheral T-cell lymphoma. Peripheral T-cell lymphoma (PTCL) is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called T-cells and natural killer cells (NK cells) (see figure for an overview of PTCL subtypes). PTCL is a type of non-Hodgkin's lymphoma (NHL). NHL affects two particular types of white blood cells: B-cells and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally.
PTCL-NOS, the most common subtype of PTCL, is aggressive and predominantly nodal. There are two morphologic variants: the T-zone lymphoma variant and the lymphoepithelioid cell variant.
- T-zone lymphoma is so named for its involvement in a specific area of the lymph node that consists of a dense accumulation of T-cells.
- Lympho-epithelioid lymphoma, also called Lennert's lymphoma, is rare and generally affects older individuals.
EATL can be classified as an extranodal peripheral T cell lymphoma, a category it shares with hepatosplenic T cell lymphoma and panniculitic T cell lymphoma.
It can be further classified in type I and II EATL.
Follicular lymphoma is a type of blood cancer. It is the most common of the indolent (slow-growing) non-Hodgkin's lymphomas, and the second-most-common form of non-Hodgkin's lymphomas overall. It is defined as a lymphoma of follicle center B-cells (centrocytes and centroblasts), which has at least a partially follicular pattern. It is positive for the B-cell markers CD10, CD19, CD22, and usually CD20, but almost always negative for CD5.
There are several synonymous and obsolete terms for this disease, such as CB/CC lymphoma (Centroblastic and Centrocytic lymphoma), nodular lymphoma and Brill-Symmers Disease.