Anaplastic large-cell lymphoma

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 four different clinical entities, all sharing the same name, which histologically share 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. Anaplastic large cell lymphoma is associated with various types of medical implants.aggressive: Sézary disease 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 four different clinical entities, all sharing the same name, which histologically share 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. Anaplastic large cell lymphoma is associated with various types of medical implants. 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 women who have textured silicone breast implants (protheses). This is known as breast implant associated anaplastic large cell lymphoma, or BIA-ALCL. It can occur as a result of breast reconstruction after a diagnosis of breast cancer or as a result of cosmetic surgery using textured silicone implants. BIA-ALCL initially occurs in the fluid contained within the scar capsule surrounding the implant, rather than the breast tissue itself. 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'). A form of ALCL is associated with implants. Textured breast implants are most commonly identified and have been the focus of research, but tibial implants, dental implants, injection port implants, gluteal implants, and gastric band placement have also been reported. Risk is highest with most strongly textured implants. Chronic inflammation is known to lead to lymphoma. It has been suggested that inflammation surrounding textured implants causes proliferation and activation of T-cells. The diagnosis of ALCL requires the examination by a pathologist of any enlarged lymph node, or any affected extranodal tissue where there the tumor is found, such as the intestine, the liver or bone in the case of systemic ALCL. For the case of cutaneous ALCL, a skin excision is recommended, and for the diagnosis of ALCL associated with breast implants, a cytologic specimen of the effusion around the breast implant or complete examination of the breast capsule surrounding the implant is required. Four forms of anaplastic large cell lymphoma are recognized: primary systemic anaplastic lymphoma kinase (ALK)-positive ALCL, primary systemic ALK-negative ALCL, primary cutaneous ALCL, and breast implant-associated ALCL. Anaplastic large cell lymphoma is characterized by 'hallmark' cells and presence for CD30. Integration of this information with clinical presentation is crucial for final classification and management of patients.