Anaplastic large-cell lymphoma

The anaplastic large-cell lymphoma ( ALCL ) is a malignant T -cell lymphoma, which is counted according to the WHO classification of the non-Hodgkin's lymphoma. The name is derived from the words, anaplasia 'and' large cell lymphoma ' from. It was first described in 1985 as an independent tumor entity.

Epidemiology

The anaplastic large-cell lymphoma occurs mainly in children and adolescents, as well as older adults can be affected. It makes up about 15 % of the child's non- Hogkin lymphomas. In pediatric patients, the median age of onset is about ten years. With a ratio of 1.8:1 boys are affected more often.

Clinic

In 60 to 70 % of patients the diagnosis is made only at an advanced stage (III or IV). Often a extranodal (outside lymph nodes) involving skin, bones and connective tissue. Occasionally, especially in the form of a neutrophilia occurs leukocytosis.

Diagnostics

The diagnosis is made by means of a biopsy. As diagnostic criteria, the WHO calls the detection of typical cells and the expression of the cytokine receptor CD30. Prognostic importance is the evidence of Anaplastic Lymphoma Kinase ( ALK ). Almost all cases of ALCL in childhood are ALK -positive.

Therapy

The treatment takes place in specialized centers within clinical studies mainly. Used are various chemotherapeutics, such as methotrexate, vinblastine, ifosfamide, cyclophosphamide, and doxorubicin. Accompanying dexamethasone is administered.

Since the end of 2012 in the European Union with the substance brentuximab vedotin is an antibody -drug conjugate (ADC ) for the treatment of systemic anaplastic large-cell lymphoma - ( sALCL ) available. Brentuximab vedotin linked an anti- CD30 antibody via an amino - linker with the cytostatic agent, monomethyl auristatin E ( MMAE ). The drug is approved to treat adults with a relapse or recurrence of a sALCL or a refractory (not responsive to the usual therapies ) sALCL.

Forecast

The prognosis in children with an event-free 5 - year survival rate of over 70 % pretty good. Adult patients with ALK -positive ALCL have a 60% better event-free survival than ALK -negative patients with 36%.