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Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma with novel FGFR1 fusion treated with pemigatinib

Year: 2025

Type: article

Abstract: A 70-year-old male presented with a 2-year history of cutaneous T-cell lymphoma (CTCL), reported as CD8 + .During this time, the patient had been treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (6 cycles); gemcitabine and oxaliplatin (3 cycles); and brentuximab vedotin; with no response.A subsequent specimen demonstrated CTCL with marked epidermotropism by CD3 + , CD4/ CD8-double negative, T-cell receptor (TCR) βF1-positive, and cytotoxic granule-associated RNAbinding protein (TIA-1)-positive cells.His lesions rapidly progressed.On physical examination, there were patches, plaques, and tumors with varying degrees of ulceration involving the back, chest, and extremities, including palms and soles (Figure 1A).A subsequent skin biopsy demonstrated an epidermotropic and folliculotropic CTCL, positive for CD8, CD5, CD7, and TIA-1 (Figure 1C-H), focally expressing granzyme B and perforin.A diagnosis of primary cutaneous CD8 + aggressive epidermotropic cytotoxic T-cell lymphoma (PCAECTL) was made.Positron emission tomography-computed tomography scan studies revealed multiple cutaneous focal uptakes and nonspecific hypermetabolic adenopathy.Flow cytometry analysis of the peripheral blood was negative for aberrant T cells.Skin-directed radiation therapy was recommended to the patient for symptomatic control.He received 6 Gy in 2 fractions to the bilateral palms (delivered with superficial 6 MeV electrons) and 6 Gy in 2 fractions to the bilateral feet (delivered with photons and bolus to treat the entire foot).Clinical improvement of the radiation-treated lesions was noted but they recurred shortly after.

Source: Blood Advances

DOI: 10.1182/bloodadvances.2024014928