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cutaneous aggressive epidermotropic CD8+ T-cell lymphoma
Monday 18 January 2010
primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma;
Definition: Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a part of the spectrum of CD30(+) cutaneous lymphoproliferative disorder, characterized by variable degrees of CD2, CD3, CD4 and CD5 expression by lymphoid cells.
PCALCLs with an expression of cytotoxic phenotype (CD8(+)) and cytotoxic proteins are uncommon.
Cutaneous CD8(+) CD30(+) lymphoproliferative lesions are difficult to classify, diagnose and may be the cause of misdiagnose.
CD8(+) PCALCL must be distinguished from CD8(+) mycosis fungoides, lymphomatoid papulosis type D and primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma.
Usually CD8(+) PCALCL is an indolent disease with a favorable prognosis, except few cases can show poor outcomes.
The high Ki-67 index points toward advanced PCALCL.
Treatment modalities include surgical excision, radiotherapy and clinical monitoring. Chemotherapy is reserved for disseminated disease.
Open references
Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma: A case report and significance of timely ancillary testing.
Kavishwar VS, Kirti C, Kush R, Sunita K, Pratiksha C.
Indian J Pathol Microbiol. 2016 Jan-Mar;59(1):131-2. doi : 10.4103/0377-4929.174881
PMID: 26960663 Free
Primary Cutaneous CD8(+) CD30(+) Anaplastic Large Cell Lymphoma: An Unusual Case with a High Ki-67 index-A Short Review.
Nasit JG, Patel SC.
Indian J Dermatol. 2015 Jul-Aug;60(4):373-7. doi : 10.4103/0019-5154.160483
PMID: 26288406 Free
A Case of Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma Misdiagnosed as Febrile Ulceronecrotic Mucha-Habermann Disease.
Sheng N, Li Z, Su W, Liu W, Zong W, Wang B, Feng S.
Acta Derm Venereol. 2016 Jan;96(1):136-7. doi : 10.2340/00015555-2155
PMID: 26039505
Paywall References
Fatal CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma with multiorgan involvement. Marzano AV, Ghislanzoni M, Gianelli U, Caputo R, Alessi E, Berti E. Dermatology. 2005;211(3):281-5. PMID: 16205076
