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

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

ALCL; Anaplastic large cell lymphoma; Anaplastic large cell T cell lymphoma; CD30-positive anaplastic large cell lymphoma; CD30-positive large cell cutaneous T-cell lymphoma; CD30-positive large cell T-cell lymphoma of the skin; Cutaneous anaplastic large cell lymphoma; large cell CD30-positive cutaneous T-cell lymphoma; Primary cutaneous CD30-positive large T-cell-lymphoma

Definition
This section has been translated automatically.

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.

Manifestation
This section has been translated automatically.

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.

Localization
This section has been translated automatically.

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

Clinical features
This section has been translated automatically.

Mostly solitary node with an average size of 2.4cm (up to 7.0cm). More rarely (14%) are multiple nodules or nodes in one body region. There is a tendency to ulceration. Spontaneous regressions and recurrences are possible (in up to 25% of patients). In the course of the disease, disseminated multiple nodules may develop (Booken N et al. 2012). Extracutaneous spread (mostly peripheral lymph nodes; furthermore: central lymph nodes, lung, CNS) is rare overall (29% at initial diagnosis).

Histology
This section has been translated automatically.

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.

Diagnosis
This section has been translated automatically.

Clinic; histological and immunohistological examination of skin and lymph nodes; molecular diagnostics (T-cell receptor rearrangement; this allows a distinction between monoclonal and polyclonal populations). S.a.u. cutaneous T-cell lymphomas. A bone marrow biopsy is not recommended in the context of tumor staging, since in one study it only yielded a positive result in 1/107 cases.

Differential diagnosis
This section has been translated automatically.

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).

Therapy
This section has been translated automatically.

  • 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.

Progression/forecast
This section has been translated automatically.

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

Literature
This section has been translated automatically.

  1. Au WY et al (2002) CD30-positive cutaneous T-cell lymphoma with concurrent solid tumour. Br J Dermatol 146: 1091-1095
  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
    : an analysis of the Mannheim Cutaneous Lymphoma Registry. J Dtsch Dermatol Ges 10:331-339.
  5. Cepeda LT et al (2003) CD30-positive atypical lymphoid cells in common non-neoplastic cutaneous infiltrates rich in neutrophils and eosinophils. Am J Surg Pathol 27: 912-918
  6. Gellrich S et al (2003) T cell receptor-gamma gene analysis of CD30+ large atypical individual cells in CD30+ large primary cutaneous T cell lymphomas. J Invest Dermatol 120: 670-675
  7. Gonzales CL et al (1991) T-cell-lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemphagocytic syndrome. Am J Pathol 15: 17-22
  8. Holloway KB et al (1992) Therapeutic alternatives in cutaneous T-cell lymphoma. J Am Acad Dermatol 27: 367-378
  9. Kempf W, Cerroni L (2016) Cutaneous lymphomas. In: Cerroni L et al Histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York S. 914-915
  10. Oregel KZ et al (2016) Complete response in a criticallyill
    patient with ALK-negative anaplastic large cell lymphoma treated with single agent brentuximab-vedotin. Expert Rev Anticancer Ther 16: 279-283.
  11. Sterry W et al (1995) Cutaneous malignant lymphomas. Z Hautkr 70: 781-788
  12. Wollina U et al (2003) Multicenter study of pegylated liposomal doxorubicin in patients with cutaneous T-cell lymphoma. Cancer 98: 993-1001

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020