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Primary Cutaneous Acral CD8-Positive T-Cell Lymphoma

MedGen UID:
1621388
•Concept ID:
C4528220
•
Neoplastic Process
Synonym: Primary cutaneous acral CD8 positive T-cell lymphoma
SNOMED CT: Primary cutaneous acral CD8 positive T-cell lymphoma (787198005)

Definition

A cutaneous T-cell lymphoma presenting as an acral (peripheral) lesion. It was originally described as originating in the ear and is usually localized to a single site. It is composed of CD8-positive neoplastic T-lymphocytes. It has an indolent clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Cutaneous Acral CD8-Positive T-Cell Lymphoma

Professional guidelines

PubMed

Long-term follow-up and management of small and medium-sized CD4(+) T cell lymphoma and CD8(+) lymphoid proliferations of acral sites: a multicenter experience.
Virmani P, Jawed S, Myskowski PL, Horwitz S, Skripnik Lucas A, Moskowitz A, Pulitzer M, Zain J, Rosen ST, Querfeld C
Int J Dermatol 2016 Nov;55(11):1248-1254. doi: 10.1111/ijd.13340. PMID: 27369070Free PMC Article

Recent clinical studies

Etiology

Recent advances in cutaneous lymphoma-implications for current and future classifications.
Goodlad JR, Cerroni L, Swerdlow SH
Virchows Arch 2023 Jan;482(1):281-298. Epub 2022 Oct 24 doi: 10.1007/s00428-022-03421-5. PMID: 36278991Free PMC Article
New developments in non-Hodgkin lymphoid malignancies.
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
Primary Cutaneous Acral CD8+ T-Cell Lymphoma-A Single Center Review of 3 Cases and Recent Literature Review.
Tjahjono LA, Davis MDP, Witzig TE, Comfere NI
Am J Dermatopathol 2019 Sep;41(9):644-648. doi: 10.1097/DAD.0000000000001366. PMID: 31433793

Diagnosis

Recent advances in cutaneous lymphoma-implications for current and future classifications.
Goodlad JR, Cerroni L, Swerdlow SH
Virchows Arch 2023 Jan;482(1):281-298. Epub 2022 Oct 24 doi: 10.1007/s00428-022-03421-5. PMID: 36278991Free PMC Article
New developments in non-Hodgkin lymphoid malignancies.
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
Atypical BCL6/GATA3+ Primary Cutaneous Acral CD8-Positive T-Cell Lymphoma: A Diagnostic Challenge.
Prieto-Torres L, Camacho-García D, Piris MÁ, Requena L, Rodríguez-Pinilla SM
Am J Dermatopathol 2021 Feb 1;43(2):137-140. doi: 10.1097/DAD.0000000000001737. PMID: 32675470
Diagnosis of T-cell lymphoid proliferations of the skin: putting all the pieces together.
Torres-Cabala CA
Mod Pathol 2020 Jan;33(Suppl 1):83-95. Epub 2019 Oct 29 doi: 10.1038/s41379-019-0397-3. PMID: 31664155

Prognosis

Atypical BCL6/GATA3+ Primary Cutaneous Acral CD8-Positive T-Cell Lymphoma: A Diagnostic Challenge.
Prieto-Torres L, Camacho-García D, Piris MÁ, Requena L, Rodríguez-Pinilla SM
Am J Dermatopathol 2021 Feb 1;43(2):137-140. doi: 10.1097/DAD.0000000000001737. PMID: 32675470
Primary Cutaneous Acral CD8+ T-Cell Lymphoma-A Single Center Review of 3 Cases and Recent Literature Review.
Tjahjono LA, Davis MDP, Witzig TE, Comfere NI
Am J Dermatopathol 2019 Sep;41(9):644-648. doi: 10.1097/DAD.0000000000001366. PMID: 31433793
Primary cutaneous acral CD8 positive T-cell lymphoma with extra-cutaneous involvement: A long-standing case with an unexpected progression.
Alberti-Violetti S, Fanoni D, Provasi M, Corti L, Venegoni L, Berti E
J Cutan Pathol 2017 Nov;44(11):964-968. Epub 2017 Sep 5 doi: 10.1111/cup.13020. PMID: 28796362

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