CD4+ small/medium-sized pleomorphic T-cell
lymphoproliferative disorder. Typical site but ulcerated lesion (ulceration occurs in around 10% of cases).
This clinical lesion was initially introduced by 2008 WHO classification as a provisional entity of primary cutaneous small/medium T-cell lymphoma. Because of its indolent behavior, the entity was modified in 2016 to primary cutaneous small/medium-sized T-cell lymphoproliferative disorder. This lymphoproliferative disorder is characterized by the expression of follicular helper T-cell markers, particularly B-cell lymphoma 6 (BCL-6), programmed cell death protein 1 (PD-1), and C-X-C motif chemokine ligand 13 (CXCL-13), while CD10 is usually negative. Molecular studies show a clonal rearrangement of T-cell receptor genes >60% of cases.
This clinical lesion was initially introduced by 2008 WHO classification as a provisional entity of primary cutaneous small/medium T-cell lymphoma. Because of its indolent behavior, the entity was modified in 2016 to primary cutaneous small/medium-sized T-cell lymphoproliferative disorder. This lymphoproliferative disorder is characterized by the expression of follicular helper T-cell markers, particularly B-cell lymphoma 6 (BCL-6), programmed cell death protein 1 (PD-1), and C-X-C motif chemokine ligand 13 (CXCL-13), while CD10 is usually negative. Molecular studies show a clonal rearrangement of T-cell receptor genes >60% of cases.
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