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Typical visible symptoms include rash-like patches, tumors, or lesions. Itching (pruritus) is common, perhaps in 20 percent of patients, but is not universal. The symptoms displayed are progressive, with early stages consisting of lesions presented as scaly patches. These lesions prefer the buttock region. The later stages involve the patches evolving into plaques distributed over the entire body. The advanced stage of mycosis fungoides is characterized by generalized erythroderma, with severe pruritus and scaling.
Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin.
While the cause remains unclear, most cases are not hereditary. Most cases are in people over 20 years of age, and it is more common in men than women. Treatment options include sunlight exposure, ultraviolet light, topical corticosteroids, chemotherapy, and radiotherapy.
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.
Lesions emerge as well-demarcated psoriasiform or hyperkeratotic patches and 36 plaques, with a central clearing and an elevated border. Pagetoid reticulosis is a very slow progressive variant of mycosis fungoides and is usually localized unlike the latter .
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.
General signs and symptoms include depression, fever, weight loss, loss of appetite, loss of hair or fur and vomiting. Lymphoma is the most common cancerous cause of hypercalcemia (high blood calcium levels) in dogs. It can lead to the above signs and symptoms plus increased water drinking, increased urination, and cardiac arrhythmias. Hypercalcemia in these cases is caused by secretion of parathyroid hormone-related protein.
Multicentric lymphoma presents as painless enlargement of the peripheral lymph nodes. This is seen in areas such as under the jaw, the armpits, the groin, and behind the knees. Enlargement of the liver and spleen causes the abdomen to distend. Mediastinal lymphoma can cause fluid to collect around the lungs, leading to coughing and difficulty breathing. Hypercalcemia is most commonly associated with this type.
Gastrointestinal lymphoma causes vomiting, diarrhea, and melena (digested blood in the stool). Low serum albumin levels and hypercalcemia can also occur.
Lymphoma of the skin is an uncommon occurrence. The epitheliotropic form typically appears as itchy inflammation of the skin progressing to nodules and plaques.
The non-epitheliotropic form can have a wide variety of appearances, from a single lump to large areas of bruised, ulcerated, hairless skin. The epitheliotropic form must be differentiated from similar appearing conditions such as pemphigus vulgaris, bullous pemphigoid, and lupus erythematosus.
Signs for lymphoma in other sites depend on the location. Central nervous system involvement can cause seizures or paralysis. Eye involvement, seen in 20 to 25 percent of cases, can lead to glaucoma, uveitis, bleeding within the eye, retinal detachment, and blindness. Lymphoma in the bone marrow causes anemia, low platelet count, and low white blood cell count.
The cancer is classified into low and high grade types. Classification is also based on location. The four location types are multicentric, mediastinal, gastrointestinal, and extranodal (involving the kidney, central nervous system, skin, heart, or eye). Multicentric lymphoma, the most common type (by greater than 80 percent), is found in the lymph nodes, with or without involvement in the liver, spleen, or bone marrow. Mediastinal lymphoma occurs in the lymph nodes in the thorax and possibly the thymus. Gastrointestinal lymphoma occurs as either a solitary tumor or diffuse invasion of the stomach or intestines, with or without involvement in the surrounding lymph nodes, liver or spleen. Classification is further based on involvement of B-lymphocytes or T-lymphocytes. Approximately 70 percent are B-cell lymphoma. Cutaneous lymphoma can be classified as epitheliotropic (closely conforming to the epidermis) or non-epitheliotropic. The epitheliotropic form is typically of T-cell origin and is also called mycosis fungoides. The non-epitheliotropic form is typically of B-cell origin.
Pagetoid reticulosis (also known as "acral mycoses fungoides", "localized epidermotropic reticulosis", "mycosis fungoides palmaris et plantaris", "unilesional mycosis fungoides", and "Woringer–Kolopp disease") is a cutaneous condition, an uncommon lymphoproliferative disorder, sometimes considered a form of mycosis fungoides.
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.
Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma is a cutaneous condition that usually presents as solitary or generalized plaques, nodules, or tumors of short duration.
The initial trauma causing the infection is often forgotten or not noticed. The infection builds at the site over a period of years, and a small red papule (skin elevation) appears. The lesion is usually not painful, with few, if any symptoms. Patients rarely seek medical care at this point.
Several complications may occur. Usually, the infection slowly spreads to the surrounding tissue while still remaining localized to the area around the original wound. However, sometimes the fungi may spread through the blood vessels or lymph vessels, producing metastatic lesions at distant sites. Another possibility is secondary infection with bacteria. This may lead to lymph stasis (obstruction of the lymph vessels) and elephantiasis. The nodules may become ulcerated, or multiple nodules may grow and coalesce, affecting a large area of a limb.
Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:
1. Generalized erythroderma
2. Lymphadenopathy
3. Atypical T-cells ("Sézary cells") in the peripheral blood
4. Hepatosplenomegaly
5. Palmoplantar keratoderma
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").
Chromoblastomycosis (also known as chromomycosis, cladosporiosis, Fonseca's disease, Pedroso's disease, phaeosporotrichosis, or verrucous dermatitis) is a long-term fungal infection of the skin and subcutaneous tissue (a chronic subcutaneous mycosis). The infection occurs most commonly in tropical or subtropical climates, often in rural areas. It can be caused by many different types of fungi which become implanted under the skin, often by thorns or splinters. Chromoblastomycosis spreads very slowly; it is rarely fatal and usually has a good prognosis, but it can be very difficult to cure. The several treatment options include medication and surgery.
Lymphatic disease is a class of disorders which directly affect the components of the lymphatic system.
Examples include Castleman's disease and lymphedema.
Diseases and disorder
Hodgkin's Disease/Hodgkin's Lymphoma
This is a type of cancer of the lymphatic system. It can start almost anywhere in the body. It is believed to be caused by HIV, Epstein-Barr Syndrome, age and family history. Symptoms include weight loss, fever, swollen lymph nodes, night sweats, itchy skin, fatigue, chest pain, coughing or trouble swallowing.
Non-Hodgkin's Lymphoma
Lymphoma is a usually malignant cancer. It is caused by the body producing too many abnormal white blood cells. It is not the same as Hodgkin's Disease. Symptoms usually include painless, enlarged lymph node or nodes in the neck, weakness, fever, weight loss, and anemia.
Lymphadenitis is an infection of the lymph nodes usually caused by a virus, bacteria or fungi. Symptoms include redness or swelling around the lymph node.
Lymphangitis
Lymphangitis is an inflammation of the lymph vessels. Symptoms usually include swelling, redness, warmth, pain or red streaking around the affected area.
Lymphedema
Lymphedema is the chronic pooling of lymph fluid in the tissue. It usually starts in the feet or lower legs. It's also a side-effect of some surgical procedures.
Lymphocytosis
Lymphocytosis is a high lymphocyte count. It can be caused by an infection, blood cancer, lymphoma, or autoimmune disorders that are accompanied by chronic swelling.
Drug-induced pseudolymphoma results from exposure to medications, which results in cutaneous inflammatory patterns that resemble lymphoma, most frequently mycosis fungoides.
Those who have Sézary disease often present with skin lesions that do not heal with normal medication. A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma. Finally, a biopsy of a skin lesion can be performed to rule out any other causes.
The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences:
1. More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome
2. Sometimes absent epidermotropism
3. Increased lymph node involvement with infiltrates of Sézary syndrome.
Malignant histiocytosis (also known as "Histiocytic medullary reticulosis") is a rare hereditary disease found in the Bernese Mountain Dog and humans, characterized by histiocytic infiltration of the lungs and lymph nodes. The liver, spleen, and central nervous system can also be affected. Histiocytes are a component of the immune system that proliferate abnormally in this disease. In addition to its importance in veterinary medicine, the condition is also important in human pathology.
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.
The sarcoma first appears as a bruise mark, a purplish discoloration or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes quickly.
Lymphadenopathy can occur in one or more groups of lymph nodes. Among 358 cases of Rosai–Dorfman disease that Rosai collected in a disease registry for which the location of lymphadenopathy was specified, 87.3% had cervical lymphadenopathy. Axillary, inguinal, and mediastinal lymphadenopathy are also found in Rosai–Dorfman disease.
The disease in the lungs is characterized by enlargement of the tracheobronchial lymph nodes and infiltration of the lungs, sometimes leading to lung lobe consolidation and pleural effusion. Signs and symptoms include cough, loss of appetite, weight loss, anemia, and difficulty breathing. Seizures and rear limb weakness can be seen. Invasion of the bone marrow can cause pancytopenia. Diagnosis requires a biopsy.
Anaplastic large-cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma involving aberrant T cells or null lymphocytes. It is described in detail in the "Classification of Tumours of the Haematopoietic and Lymphoid Tissues" edited by experts of the World Health Organisation (WHO). The term anaplastic large cell lymphoma (ALCL) encompasses at least 4 different clinical entities, all sharing the same name, and histologically have also in common the presence of large pleomorphic cells that express CD30 and T-cell markers. Two types of ALCL present as systemic disease and are considered as aggressive lymphomas, while two types present as localized disease and may progress locally.
Its name derives from anaplasia and large-cell lymphoma.
Accumulation of histiocytes may occur outside of lymph nodes. The most common sites of extranodal disease in Rosai's registry were skin, nasal cavity/paranasal sinuses, soft tissue, eyelid/orbit, bone, salivary glands, and central nervous system.
The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.
Often, this disease evolves from a precursor lesion, usually a dysplastic nevus. Otherwise it arises in previously normal skin. A prolonged radial growth phase, where the lesion remains thin, may eventually be followed by a vertical growth phase where the lesion becomes thick and nodular. As the risk of spread varies with the thickness, early SSM is more frequently cured than late nodular melanoma.
The microscopic hallmarks are:
- Large melanocytic cells with nest formation along the dermo-epidermal junction.
- Invasion of the upper epidermis in a pagetoid fashion (discohesive single cell growth).
- The pattern of rete ridges is often effaced.
- Invasion of the dermis by atypical, pleomorphic melanocytes
- Absence of the 'maturation' typical of naevus cells
- Mitoses