Primary cutaneous cd30 positive large cell t cell lymphoma C86.6

Malignant T-cell lymphoma of the skin, consisting of large cells with anaplastic, pleomorphic or immunoblastic cytomorphology, which contains > 75% of the CD30 antigen (activation marker from the group of nerve growth factor receptors; see below growth factors) is expressed.

The mean age of onset of the disease is about 57 years. Men are affected in a ratio of 6.7:3.4 compared to women.

Preferably on the upper half of the body. In 43% of cases on the arms.

Mostly solitary nodule with a mean size of 2.4cm (to 7.0cm). Rarer (14%) are multiple nodules or nodules in one body region. There is a tendency to ulceration. Spontaneous regression and recurrence are possible (in up to 25% of patients). Disseminated, multiple nodules may develop as the disease progresses (Booken N et al 2012). Extracutaneous spread (mostly peripheral lymph nodes; furthermore: central lymph nodes, lung, CNS) is rare overall (29% at initial diagnosis).

Regarding the systemic forms see below. Anaplastic large cell lymphoma ALK-positive (ALCL, ALK+)

Characteristic are predominantly large-cell, diffuse or nodular, anaplastic, rarely pleomorphic or immunoblastic infiltrates of the skin. Moderate to missing epidermotropy.

Immunohistological criteria: > 75% of tumor cells express the CD30 antigen; small lymphocytes are negative. T-cell patterns are usually detectable. Some of the tumours (29%) show an antigen loss of the pan-T-cell marker(CD3), 14% of the T-cell marker CD4. CD8 cells are only found in a very small proportion (<5% CD20+ cells). The proliferative activity (MIB antibodies) is increased in all patients. It is usually found in 55% of tumour cells. EMA (= epithelial membrane antigen) is only very sporadically reactive in this type of lymphoma (Booken N et al. 2011)

Cytogenetically, a monoclonal rearrangement of T cell receptor genes (TZR gamma genes) can be detected in the majority of cases.

Clinic.

histological and immunohistological examination of skin and lymph nodes.

molecular diagnostics (T-cell receptor rearrangement.

This allows differentiation between monoclonal and polyclonal populations). See also Cutaneous T-cell lymphomas. Bone marrow biopsy is not recommended in the context of tumor staging, since in one study this yielded a positive result in only 1/107 cases.

Clinical:

• Mycosis fungoides: The clinical picture of cutaneous anaplastic large cell (T-cell) lymphoma can be distinguished from the tumor stage of Mycosis fungoides by the long lasting "precursor situation" of Mycosis fungoides.
• B-cell lymphoma: A tendency to ulcer formation rather speaks against B-cell lymphoma
• Nodal anaplastic large cell lymphoma: Detection of extracutaneous involvement. The local clinical findings are not distinguishable.

Histological:

• Nodal anaplastic large cell lymphoma: Differential diganosis usually only succeeds by additional clinical findings (proof of extracutaneous involvement).
• Lymphomatoid papulose type C: Differential diagnosis is usually only possible with the additional clinical findings (typical recurrent course with long latencies; no extracutaneous infestation).
• B-cell lymphomas: excluded by the proving immunological phenotype (B-cell pattern).

• In case of isolated tumor excision or irradiation: fractionated radiotherapy (2-3 times/week, GD 25-30 Gy, ED 2-5 Gy).
• For multifocal lesions with regressions: radiotherapy (see above).
• For disseminated occurrence: chemotherapy or radiation therapy:
  • chemotherapy:
  • Methotrexate: Treatment with low-dose methotrexate should be carried out first. In case of recurrence or therapy failure, a less aggressive regimen (e.g. budding regimen) should be applied before more aggressive combinations are used. Methotrexate, bud regimen, CHOP regimen, COPBLAM regimen are used. For handling, side effects and laboratory controls see there. See also cytostatics.
  • Radiation therapy: Alternatively, for disseminated tumours, whole body radiation with fast electrons (GD: 30-36 Gy, ED: 2 Gy).
  • Brentuximab Vedotin is an antibody-drug conjugate (ADC) with a monoclonal antibody directed against human CD30 antigen. The antibody is covalently bound to 3-5 molecules of the cytostatic drug monomethylauristatin E. See below for the results of the pivotal study Brentuximab Vedotin.

In contrast to the CD30-negative large cell T-cell lymphomas rather favorable; 10-year survival rate about 90%.

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2. Beljaads RC et al (1993) Primary cutaneous CD 30-positive large cell lymphoma. Definition of a new type of cutaneous lymphoma with favourable prognosis. An European multicenter study on 47 cases. Cancer 71: 2097-2104
3. BennerMF et al (2008) Bone marrow examination has limited value in the staging of patients with anaplastic large cell lymphoma (ALCL) first presenting in the skin. Retrospectively analysis of 107 patients. Br J Dermatol 159: 1148-1151
4. Booken N et al (2012) Clinical spectrum of primary cutaneous CD30-positive anaplastic large celllymphoma
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