CD4 + primary cutaneous small/medium-sized pleomorphic T-cell lymphoma: a retrospective case series and review of literature

Abstract

CD4 + primary cutaneous small/medium-sized pleomorphic T-cell lymphoma (CD4 + PCSM-TCL) is a rare T-cell lymphoma associated with a favorable prognosis. A retrospective study of 23 patients with CD4 + PCSM-TCL as defined by World Health Organization-European Organisation for Research and Treatment of Cancer (WHO-EORTC) and WHO classifications was conducted. Median age was 63 years. The head and neck were the most commonly affected locations, followed by the trunk. Two patients had evidence of systemic involvement at relapse. All tumors were CD3 + and CD4+. CD5 and CD7 loss occurred in 52% and 84%, respectively. The median follow-up was 33.6 months. Eleven patients had excisional biopsy only, six had localized radiotherapy and two received excision and localized radiation. Cytotoxic chemotherapy and localized radiation were used in one patient with aggressive and invasive features. All patients had a complete remission but one developed systemic involvement. Our case series demonstrates that CD4 + PCSM-TCL is an indolent T-cell lymphoma that can be treated with local modalities and raises the question of its current classification as a lymphoma.

Cutaneous Small/Medium CD4+ Pleomorphic T-Cell Lymphoma–Like Nodule in a Patient With Erythema Chronicum Migrans

“…6,7 TCR genes are clonally rearranged in most of the cases. 4,6,7 However, the well-established phenomenon of T-cell clonality in benign and reactive conditions makes this an unreliable distinguishing factor between cutaneous lymphoid hyperplasia and true lymphoma. 4 Clonality is also not a reliable predictor of aggressive course.…”

“…4,6,7 However, the well-established phenomenon of T-cell clonality in benign and reactive conditions makes this an unreliable distinguishing factor between cutaneous lymphoid hyperplasia and true lymphoma. 4 Clonality is also not a reliable predictor of aggressive course. 8 In our case, the TCR-g results demonstrated a single peak in the V9-Jp region that met our laboratory's criteria for a clonal peak, which was reproducibility in duplicate polymerase chain reactions and a ratio of more than 5:1 comparing with the background of polyclonal amplification.…”

“…2,3 Even in the presence of apparently worrisome pathologic features, such as dense infiltrates of lymphocytes with atypical cytology, invasion of adnexal structures, ulceration, aberrant phenotype, positive T-cell clonality, and multiple lesions, CD4+ PCSM-TCL has an indolent course and rare recurrence rate. [3][4][5] Thus, its classification as a lymphoma has been questioned.…”

Recent advances in primary cutaneous T-cell lymphoma

“…Roughly 3% of CTCLs are comprised of other rare subtypes: subcutaneous panniculitis-like T-cell lymphoma (SPTCL) includes those with the a/b T-cell phenotype [4], whereas lesions with gamma-delta (gd) T-cell phenotype are subcategorized as primary cutaneous gd T-cell lymphoma [5]. Primary cutaneous CD4þ small/medium-sized pleomorphic T-cell lymphoma [6], extranodal NK/T-cell lymphoma [7], aggressive epidermotropic cutaneous CD8þ lymphoma [8], angioimmunoblastic T-cell lymphoma [9], hydroa vacciniforme-like cutaneous T-cell lymphoma [10], and peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) are other rarer subtypes of CTCL [11]. The clinical picture and prognosis in CTCL varies widely based on disease subtype and stage (adapted from the WHO-EORTC classification of CTCL based on clinical course) [12].…”

Primary cutaneous CD4+ small-to-medium-sized pleomorphic T-cell lymphoma: a rare case report of infant

“…Neoplastic cells have a CD3+/CD4+/CD8(−)/CD30(−) immunophenotype, with no expression of CD56 and TIA-1. Diminished or absent expression of CD7, CD2 or CD5 is seen in a few cases 3. The proliferation fraction is low to intermediate (10%–30% of cells) with the Ki-67 immunostain.…”