PMC8189774_01
Image of the patient's chest and abdomen with erythroderma.
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An 82-year-old woman presented to our hospital because of erythroderma with scattered invasive erythemas, nodules, and tumors on the face, trunk, and extremities (Figure 1).
May-Giemsa staining of a blood smear showing abnormal cells with cerebriform nuclei (x1,000).
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PB analysis revealed a white blood cell count of 14,730/microL (reference range, 3.3-8.6 x 103/microL), with 28% abnormal cells containing cerebriform nuclei (Figure 2), a hemoglobin level of 12.2 g/dL (11.6-14.8 g/dL), and a platelet count of 311 x 103/microL (158-348 x 103/microL).
Hematoxylin-eosin (HE) staining and immunohistochemical staining of skin biopsy specimens (x200). HE staining shows the infiltration of abnormal lymphocytes in the superficial dermis (upper left). Immunohistochemical staining shows abnormal lymphocytes are positive for CD3 (upper middle) and CD4 (upper right), but negative for CD7 (lower left), CD8 (lower middle), and CD30 (lower right).
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Skin biopsy specimens from a tumor on the left arm showed the infiltration of abnormal lymphocytes with medium- to large-sized nuclei containing convolutions in the superficial dermis (Figure 3).
Results of multicolor flow cytometry of PB cells. After discriminating for dead cells and CD45- cells, the remaining cells were gated on CD3+, and then three T-lymphocyte fractions, CD4+CD8-, CD4-CD8-, and CD4-CD8+, were analyzed for CD7 and CD26 expression. Percentages of each cell fraction are indicated in the plot areas. Abbreviations: FSC-A, forward scatter area; SSC-A, side scatter area; 7-AAD, 7-aminoactinomycin D.
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T cells, 84.0% were CD4-CD8-, 10.8% were CD4+CD8-, and 4.8% were CD4-CD8+ (Figure 4). CD4+CD8- T cells expressed CD7 and/or CD26 similar to normal helper T cells, whereas CD4-CD8- T cells had a diminished expression of CD7 and lacked CD26 expression, consistent with the Sezary cell phenotype. T-cell receptor (TCR) beta-chain gene clonality assays were performed in the PB and skin samples in accordance with the
Results of TCR beta-chain gene clonality assays. PCR products were produced and analyzed in accordance with the BIOMED-2 guidelines. The results of capillary electrophoresis of PCR products derived from PB (left) and skin (right) samples are shown. (a), (b), and (c) indicate the data of Vbeta + Jbeta1/2, Vbeta + Jbeta2, and Dbeta + Jbeta1/2, respectively. In each figure, an upper figure shows data from negative control and a lower shows data from sample. The vertical line shows fluorescence intensity, the horizontal line shows fragment size, red arrows show clonal peak, and blue arrows show non-specific peak.
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Tokyo, Japan). Both PB and skin samples showed a clonal peak in Vbeta + Jbeta1/2 and Dbeta + Jbeta1/2, and the size of those PCR products was completely matched between both specimens as 273 bp in Vbeta + Jbeta1/2 and 296 bp in Dbeta + Jbeta1/2 (Figure 5). The results suggested that tumor cells in PB and skin were clonally identical, consistent with the pathological definition of SS.