Performance of the SCORTEN during the first five days of hospitalization to predict the prognosis of epidermal necrolysis.

The SCORTEN, calculated within 24 hours of admission, is a severity-of-illness score validated for toxic epidermal necrolysis and Stevens-Johnson syndrome. Our purpose was to assess the performance of successive SCORTEN during the first 5 days of hospitalization and to determine the influence of admission delay. Charts of 144 patients aged 46.8 years (+/-19.7), admitted to our department (1993-2003) with Stevens-Johnson syndrome or toxic epidermal necrolysis, were reviewed. Successive SCORTEN were compared between deceased patients (n = 28, 19.4%) and survivors (n = 116). The performance of the score (calibration, discrimination) was assessed on days 1-5. All seven SCORTEN variables, on days 1-5, were associated with a higher mortality rate. The SCORTEN rose slightly during hospitalization, with a significant difference between days 1 and 4 (<0.05). Performance of the SCORTEN was good on each day, but slightly better on day 3. The areas under the receiver-operating characteristic curves were above 80%. The admission delay did not differ between deceased patients and survivors. Delay-adjusted SCORTEN was close to the crude SCORTEN. The SCORTEN performance during the first 5 days of hospitalization was excellent, and at its best on day 3. We recommend to compute again the SCORTEN on day 3. The admission delay did not influence prognosis or SCORTEN.

CD158k/KIR3DL2 is a new phenotypic marker of Sezary cells: relevance for the diagnosis and follow-up of Sezary syndrome.

CD158k molecules belong to the family of killer cell immunoglobulin-like receptors (KIR) that are expressed on a minor population of circulating NK and CD8+ T lymphocytes. Here, we report a strong positive correlation between the percentage of CD158k+ blood lymphocytes analyzed by flow cytometry and the percentage of atypical circulating cells (Sezary cells) determined by cytomorphology in a large group of patients with Sezary syndrome. Moreover, we show that circulating CD4+CD158k+ lymphocytes correspond to the malignant clonal cell population. Our findings suggest that the CD158k marker could be a useful tool for the evaluation of the circulating tumoral burden and the follow-up of patients with Sezary syndrome.

Sezary syndrome cells unlike normal circulating T lymphocytes fail to migrate following engagement of NT1 receptor.

Circulating malignant Sezary cells are a clonal proliferation of CD4+CD45RO+ T lymphocytes primarily involving the skin. To study the biology of these malignant T lymphocytes, we tested their ability to migrate in chemotaxis assays. Previously, we had shown that the neuropeptide neurotensin (NT) binds to freshly isolated Sezary malignant cells and induces through NT1 receptors the cell migration of the cutaneous T cell lymphoma cell line Cou-L. Here, we report that peripheral blood Sezary cells as well as the Sezary cell line Pno fail to migrate in response to neurotensin although they are capable of migrating to the chemokine stromal-cell-derived factor 1 alpha. This is in contrast with normal circulating CD4+ or CD8+ lymphocytes, which respond to both types of chemoattractants except after ex vivo short-time anti-CD3 monoclonal antibody activation, which abrogates the neurotensin-induced lymphocyte migration. Furthermore, we demonstrate that neurotensin-responsive T lymphocytes express the functional NT1 receptor responsible for chemotaxis. In these cells, but not in Sezary cells, neurotensin induces recruitment of phosphatidylinositol-3 kinase, and redistribution of phosphorylated cytoplasmic tyrosine kinase focal adhesion kinase and filamentous actin. Taken together, these results, which show functional distinctions between normal circulating lymphocytes and Sezary syndrome cells, contribute to further understanding of the physiopathology of these atypical cells.

Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae.

BACKGROUND: Acral vascular syndromes associated with malignancy have rarely been reported. OBJECTIVE: Our purpose was to assess the clinical and evolving features of paraneoplastic acral vascular syndromes. PATIENTS AND METHODS: Two cases of paraneoplastic gangrene are described and analyzed together with previously reported cases identified by a MEDLINE search. RESULTS: Among the 68 patients identified, 40 had gangrene, 16 had acrocyanosis, and 12 had Raynaud's phenomenon. The male to female ratio was 0.89; median age was 59 years. Fingers were affected in 94%. Adenocarcinomas were the predominant associated malignancies (41%), and metastases were observed in 41%. The acral vascular syndromes in 48% of the patients definitively regressed after tumor treatment. Forty-four percent of the patients died within 2 years. A favorable cutaneous outcome was obtained with prostacyclin infusions in 6 patients. CONCLUSION: A neoplastic origin of acral vascular syndrome should be considered in elderly patients, especially men, in the absence of usual causative conditions.