+ T-cell lymphoma, and six CD30 + lymphoproliferative disorders (three lymphomatoid papulosis and three anaplastic large-cell lymphomas). In addition, 43 samples from patients with ID were identified.The performance of the HTS TRB technique for CTCL diagnosis was analysed using receiver operating characteristic (ROC) curves on 101 cases of CTCL and 43 of ID. As shown in Figure 1(a, b), the TCF showed the highest diagnostic performance, with an area under curve of 0Á96, vs. 0Á93 for RPF. TCF and RPF values by definite diagnostic categories are shown in Figure 1(c, d).With 5% and 25% TCF thresholds, the specificities for CTCL diagnosis were 95% and 100%, and sensitivity 89% and 50%, respectively. Such a high specificity allows early identification of CTCL in difficult cases. One of our patients had a skin biopsy showing spongiotic dermatitis 2 years before a definite SS diagnosis. Retrospectively, HTS performed at that time already showed a TCF of 59% in skin, which corresponds on the ROC curve to a specificity of 100% and a sensitivity of 84%. Only two patients without CTCL (dermatitis and prurigo) had a TCF > 5% in skin: one died of a stroke and the other is still alive 2 years after the diagnosis. There was a significant Spearman correlation between TCF and blood tumour burden in patients with SS (absolute numbers of CD4 + CD26 À T cells, r = 0Á59, P < 0Á001; CD4 + CD7 À , r = 0Á45, P = 0Á01; CD4 + CD158k + , r = 0Á4, P = 0Á03). Clonality analyses in skin and blood were performed by HTS TRB and PCR of TRG in nine patients with SS. Both techniques identified an identical T-cell clone in all blood-skin couples of samples, except in one blood sample in which a dominant T-cell clone was identified by HTS TRB, identical to the skin, but no dominant Tcell clone was identified by PCR.In conclusion, the TCF of TRB determined by HTS was a robust criterion for CTCL diagnosis, and especially useful for early-stage MF, with 95% specificity and 89% sensitivity at the 5% threshold. This compares with 88% specificity and 72% sensitivity using PCR of TRG. 8 This threshold is identical to that used in the study by Sufficool et al., 7 who used HTS of TRG and found 85% sensitivity for CTCL diagnosis (specificity was not analysed because no cases of ID were included). Rea et al. 8 used HTS TRB clonality score and found 100% specificity and 68% sensitivity at the 0Á175 cutoff. In our dataset, clonality score was less accurate than TCF. Further multicentre prospective studies are needed to validate international criteria for T-cell clonality analysis by HTS of TCR genes in the diagnosis of CTCL.
in EMCD 2,4,5 and immune complexes consisting of IgM, IgA, C3, and fibrin deposited in cutaneous vasculature found in EM. 4 Limitations of this systematic review include small sample sizes, lack of high-quality randomized controlled trials, and lack of follow-up data. In addition, confirmation of EMCD in all included cases is difficult to determine. However, positive patch testing confirmed 95.1% (n = 116/122) of allergens, EM-like histology was confirmed by biopsy in 92.7% (n = 38/41) of patients, and targeted lesions were reported in 78.5% (n = 84/107) of patients. Despite these limitations, our findings provide important conclusions to guide EMCD management, showing that 99.0% cases (n = 98/99) of EMCD achieved CoR with allergen withdrawal or allergen withdrawal and corticosteroids.
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