Aggressive cytotoxic epidermotropic cd8-positive t-cell lymphoma C84.5

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 08.01.2023

Synonym(s)

CD8-positive cutaneous T-cell lymphoma; CD8-positive T-cell lymphoma of the skin; Cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma; Cutaneous cytotoxic T-cell lymphoma; Cytotoxic cutaneous T-cell lymphoma; Cytotoxic T-cell lymphoma of the skin

Definition
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Very rare, primary T-cell lymphoma of the skin, in which the tumor cells by definition exhibit the phenotype of cytotoxic T-cell with expression of CD3 and CD8 (see below for an overview of lymphoma, cutaneous T-cell lymphoma, cytotoxic). CD8+ CTCL is characterized by its own clinical and immunohistological characteristics and a well defined aggressive course.

Clinical features
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Generalized, very rapidly spreading skin changes with red, little itchy papules, plaques and nodules, which ulcerate at a certain size. There is a tendency to early metastasis into extracutaneous organs such as lungs, testes, CNS, oral mucosa. In contrast to other cutaneous T-cell lymphomas, the lymph nodes usually remain free of tumor infiltrates.

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Histology
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Histology: Predominantly image as in mycosis fungoides or pagetoid reticulosis with band-shaped, diffuse infiltrate of small, medium or large immunoblastic pleomorphic, distinctly epidermotropic T cells. The epidermis is acanthotic with different, but mostly very dense lymphocytic infiltrates.

Immunohistology: High Ki 67 positive marker index (proliferation marker). Positive marker for CD7, CD8, CD45RA as well as TIA-1, Granzyme B, Perforin. Negative for CD2- (= pan T-cell marker).

Diagnosis
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Clinic with rapidly generalized cutaneous infestation. Histology with typical immunohistological pattern: positive labelling for CD7, CD8, CD45RA as well as TIA-1, Granzyme B, Perforin. Negative for CD2- (= pan T-cell marker).

Differential diagnosis
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Cutaneous T-cell lymphoma of other provenance (see lymphoma, cutaneous T-cell lymphoma below).

Mycosis fungoides: Clinically varied and variable picture (time interval from first symptoms to diagnosis: 4.2 years!). Skin symptoms develop very slowly (months to years). Systemic involvement is only detectable after a longer clinical course. Typical clinical course of lymphoma in 3 stages.

Pagetoid reticulosis "disseminated type" (Ketron and Goodman): Disseminated, reddened, rapidly growing, scaly plaques and nodules, especially in older men. Tendency to generalization.

Notice. These were probably T-cell lymphomas that must be classified as CD8+ cutaneous T-cell lymphoma.

Therapy
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Apparently, there is a clear resistance to conventional therapy modalities such as PUVA, interferon-alpha or classical retinoids. Even under aggressive therapies such as polychemotherapy or allogenic bone marrow transplantation, complete remissions cannot be achieved. There is a report on full remission with bexarotene as well as whole body irradiation with linear accelerator (GD 20 Gy). The survival time from the time of diagnosis is between 15 and 50 months in the cases known so far.

Progression/forecast
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Bad prognosis. The mean survival time is about 32 months.

Literature
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Berti E et al (2009) Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. At J cathode 155: 483-492
Gormley RH et al (2010) Primary cutaneous aggressive CD8+ T cell lymphomas. Am J Acad Dermatol 62: 300-307
El-Shabrawi-Caelen L et al (2000) The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Sem Cutan Surg 19:118-123
Santucci M et al (2003) Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer 97: 610-627