Pagetoid reticulosis positive for cytotoxic CD20 and CD30: A case report.

Pagetoid reticulosis is an indolent cutaneous T-cell lymphoma and presents as erythema or plaque with a well-defined border on the distal areas of the extremities. Immunophenotypic studies show that in most cases, neoplastic lymphocytes are positive for CD4, whereas CD20 and CD30 double positivity was rarely reported. In this paper, we report an 80-year-old woman who presented with erythema on the extremities for 3 years. Skin biopsy on the right forearm was performed. Histopathologically, the erythematous lesions were characterized by atypical lymphocytes with significant epidermotropism. Immunohistochemical staining showed high proliferation as evidenced by high Ki-67 index and that the tumor cells were positive for CD20 and CD30 but negative for CD7 and CD56. The patient was treated with one cycle of radiotherapy and is currently doing well.

Pagetoid reticulosis tumor cells with double expression of TCRγδ and TCRαß: an off-target phenomenon or genuine expression?

Pagetoid reticulosis (PR) is a low-grade primary cutaneous T-cell lymphoma showing localized patches or plaques with an intrapeidermal proliferation of neoplastic T-cells with heterogeneous immunophenotype. We describe a 73-year-old woman with a 8-year history of gluteal lesions of PR, whom large blast cells were CD4/CD8 double negative T-cells with an activated cytotoxic profile. The case was investigated using a broad panel of monoclonal antibodies including TCRγM1, a new available antibody that recognizes the γ chain subunit of the T-cell receptor (TCR) in formalin-fixed paraffin-embedded tissue. Large blast cells were simultaneously positive for TCRαß and TCRγδ with an activated cytotoxic phenotype. It is worldwide accepted the mutual exclusive expression of TCRαß and TCRγδ but six different studies, dealing with TCRγδ expression in various types of extra-nodal lymphomas, reported cases whom tumor cells expressed simultaneously TCRαß and TCRγδ. Our data and those of similar reports, suggest the possibility of existence of a subset of extra-nodal T-cell lymphomas showing simultaneous expression by tumor cells of TCRγδ and TCRαß with an immunoprofile consistent with an origin from TCRγδ+ T lymphocytes. This unusual subset has preferential, but not exclusive, skin localization and variable epidermotropism.

Treatment of pagetoid reticulosis with intensity modulated radiation therapy.

Pagetoid reticulosis, also known as Woringer-Kolopp disease, is a rare variant of cutaneous T-cell lymphoma usually presenting as an isolated patch or plaque on the extremities. Immunohistochemical staining of T-cell markers can be variable, but as the name implies the epidermotropic lymphocytes consistently display a "pagetoid" appearance. The following case demonstrates clearance of this condition with intensity modulated radiation therapy, whereas most cases are managed with electron beam therapy if radiation therapy is implemented.

[Pagetoid reticulosis in a patient with mycosis fungoides. Successful therapy with localized electron beam irradiation]. / Pagetoide Retikulose bei einem Patienten mit Mycosis fungoides. Erfolgreiche Therapie mit lokalisierter Elektronenbestrahlung.

Pagetoid reticulosis is a mycosis fungoides subtype. It is characterized by a prominent epidermotropism, localized lesions, slow progression and benign prognosis. We report on a 67-year-old patient with a history of classic mycosis fungoides, who developed pagetoid reticulosis on the right heel. Local electron beam therapy induced a complete remission, with no recurrence over three months of follow-up.

CD4/CD8 double negative pagetoid reticulosis: a case report and literature review.

Pagetoid reticulosis is an indolent primary cutaneous T-cell lymphoma. It typically presents as a solitary and slowly growing patch or plaque on the extremity, histologically characterized by an acanthotic epidermis infiltrated with atypical lymphocytes. Here, we present histological, immunophenotypical and molecular findings of a 29-year-old Jamaican man with bilateral wrist plaques. Histology showed marked acanthosis, hyperkeratosis and an intraepidermal infiltration consisting of large atypical lymphocytes. Immunohistochemical stains showed CD3 and CD5 positive T cells with significant loss of CD7, double negative CD4 and CD8 and strong positive CD30. Molecular analysis showed a monoclonal T-cell receptor (TCR) gamma gene rearrangement. Review of the literature confirms that the immunophenotype of pagetoid reticulosis is variable with decreasing frequency of CD8+ cytotoxic/suppressor T cell, CD4+ helper T cell and least commonly CD4/CD8 double negative phenotypes. Although CD4/CD8 double negative phenotype appears to be associated with higher proliferation index, it does not appear to confer prognostic significance.