Primary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorder

MedGen UID:: 237165
•Concept ID:: C1371159
•: Neoplastic Process

Synonyms:	Primary cutaneous CD30 antigen positive T-cell lymphoproliferative disorder; Primary cutaneous CD30+ T-cell lymphoproliferative disorder; Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorder
SNOMED CT:	Primary cutaneous CD30+ T-cell lymphoproliferative disorder (128804002); Primary cutaneous CD30 antigen positive T-cell lymphoproliferative disorder (128804002); Primary cutaneous CD30 antigen positive T-cell lymphoproliferative disorder (896745008)

Definition

This entity represents a spectrum of lymphoproliferative disorders characterized by CD30 (Ki-1)-positive cutaneous T-cell infiltrates. The two ends of the spectrum include lymphomatoid papulosis (benign end) and primary cutaneous anaplastic large cell lymphoma (malignant end). Borderline lesions are also included in this spectrum. (WHO, 2001) [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPrimary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorder

Disorder by Site
- Hematopoietic and Lymphoid System Disorder
  - Hematologic disorder
    - Hematopoietic and lymphoid cell neoplasm
      - Cutaneous Hematopoietic and Lymphoid Cell Neoplasm
        Cutaneous Mature T-Cell and NK-Cell Neoplasm
        Primary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorder
        Borderline Primary Cutaneous CD30-Positive T-Cell Lymphoproliferative Disorder
        Anaplastic Large Cell Lymphoma, Lymphomatoid Papulosis-Like Histology
        Type C Lymphomatoid Papulosis
        Primary cutaneous anaplastic large cell lymphoma
        Adult Primary Cutaneous Anaplastic Large Cell Lymphoma
        Childhood Primary Cutaneous Anaplastic Large Cell Lymphoma
        Recurrent Primary Cutaneous Anaplastic Large Cell Lymphoma
        Refractory Primary Cutaneous Anaplastic Large Cell Lymphoma
        Type A Lymphomatoid Papulosis

Professional guidelines

PubMed

Brentuximab-vedotin in combination with cyclophosphamide, doxorubicin, prednisolone for the treatment of aggressive CD30-positive cutaneous T-cell lymphomas.

Ribereau-Gayon E, Donzel M, Pham F, Romain-Scelle N, Perier-Muzet M, Balme B, Traverse-Glehen A, Ghesquières H, Dalle S
Leuk Lymphoma 2023 Jul-Aug;64(8):1424-1432. Epub 2023 May 31 doi: 10.1080/10428194.2023.2216820. PMID: 37255021

Cutaneous CD30-positive T-cell lymphoproliferative disorders-clinical and histopathologic features, differential diagnosis, and treatment.

Kempf W, Kerl K, Mitteldorf C
Semin Cutan Med Surg 2018 Mar;37(1):24-29. doi: 10.12788/j.sder.2018.001. PMID: 29719017

Brentuximab vedotin therapy for CD30-positive cutaneous T-cell lymphoma: a targeted approach to management.

Scarisbrick JJ
Future Oncol 2017 Nov;13(27):2405-2411. Epub 2017 Aug 14 doi: 10.2217/fon-2017-0263. PMID: 28805086

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Recent clinical studies

Etiology

Prevalence of T-cell antigen losses in mycosis fungoides and CD30-positive cutaneous T-cell lymphoproliferations in a series of 153 patients.

Wechsler J, Ingen-Housz-Oro S, Deschamps L, Brunet-Possenti F, Deschamps J, Delfau MH, Calderaro J, Ortonne N
Pathology 2022 Oct;54(6):729-737. Epub 2022 May 14 doi: 10.1016/j.pathol.2022.02.008. PMID: 35577607

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