Anaplastic large cell lymphoma

Anaplastic large cell lymphoma - very high mag

Anaplastic large cell lymphoma (ALCL) is a rare type of Non-Hodgkins lymphoma. Lymphoma is a cancer of the lymph system, part of our immune system. Non-Hodgkins lymphoma involves abnormal growth of white blood cells, either T cells or B cells. Anaplastic large cell lymphoma is an aggressive cancer that usually involves the T-cells. Cancer cells in ALCL can be identified by their appearance under the microscope and by the presence of a tumor maker called CD30 or Ki-1.

Types

There are two types of ALCL, a type that affects mainly the skin (cutaneous ALCL) and a type that affects other body organs (systmic ALCL). Systemic ALCL also has two types, ALK-positive (anaplastic lymphoma kinase) and ALK-negative. ALK-positive ALCL occurs more often in children and young adults. ALK-negative ALCL tends to occur in older adults.

symptoms

The symptoms of cutaneous ALCL include red skin lesions that break open and do not heal. Sometimes cutaneous ALCL is also found in the lymph nodes . The symptoms of systemic ALCL include fever, night sweats, and weight loss (B symptoms). The cancer can be found in the bone, soft tissue , spleen, liver and skin.

cause

The cause of ALCL is unknown. One type of ALK-negative ALCL has been associated with breast implants.

Diagnosis

ALCL is diagnosed by a biopsy of the tumor or abnormal skin and examination of the tumor cells under a microscope. Additional testing including PET scans,CTscans ,MRI and a bone marrow biopsy can tell doctors if the cancer has spread to other organs.

Treatment

ALCL is treated using chemotherapy , and a stem-cell transplant for people with more aggressive cancer or for ALCL that has come back. In addition, there are newer treatments that target the cancer cells directly and have been very effective. In general, ALK-positive ALCL has a better long-term outlook than ALK-negative ALCL

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Brentuximub vedotin (Brand name: Adcetris)brentuximab vedotin (Adcetris) was approved for the treatment of adult patients with previously untreated systemic anaplastic large cell  lymphoma (sALCL) or other CD30-expressing peripheral T-cell lymphomas (PTCL), including angioimmunoblastic T-cell lymphoma and PTCL not otherwise specified, in combination with cyclophosphamide, doxorubicin, and prednisone .

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NIH genetic and rare disease info

• NIH info on Anaplastic large cell lymphoma

Anaplastic large cell lymphoma is a rare disease.