In chemotherapy-treated NSCLC patients, EGFR gene copy number was positively associated with protein level but none of the features were predictive for either treatment response or survival.

Alpha(1)-antitrypsin deficiency, a relatively frequent mutation in the population, is associated with the development of panlobular emphysema and liver cirrhosis. The deficiency is in rare cases associated with the development of panniculitis, and very differentiated clinical courses have been reported in the literature. We report a case of panniculitis in a patient with alpha(1)-antitrypsin deficiency and describe briefly the pathophysiology of the disease and current treatment possibilities.

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7193 Background: High EGFR gene copy number by fluorescence in situ hybridization (FISH) predicts response and survival benefit in non small-cell lung cancer (NSCLC) patients (pts) treated with EGFR tyrosine-kinase inhibitors, but its prognostic value remains debated. We aimed to evaluate the association of EGFR FISH, EGFR immunohistochemistry (IHC) and prognosis in NSCLC pts treated with chemotherapy alone. Methods: 85 pts treated with platinum-containing chemotherapy (median follow up of 15 months [range: 2–29 months]) were included in the study. There were 47% females, 35% of pts with performance status (PS) 0, 53% PS = 1 and 12% PS = 2, 6% of never-smokers. Median age was 62 years (range: 41–80 years). Stage I-IIIA was diagnosed in 7%, stage IIIB - 44% and stage IV - 48% pts. Adenocarcinoma was the most common histology (51% pts). EGFR FISH was performed using LSI EGFR SpectrumOrange/CEP 7 SpectrumGreen probe and IHC using DAKO PharmDx kit. Results: FISH results were available in 79 pts (93%), and EGFR FISH-positive tumors (high polysomy or gene amplification) were found in 28 pts (35%). IHC results were available in 81 pts (95%) and 25 pts (31%) were scored as positive (staining index ≥200). Distribution of clinical characteristics did not differ according to either FISH or IHC result. FISH-positive pts had higher EGFR IHC staining indices as compared with FISH-negative pts (median 160 vs. 60, p = 0.005, Mann-Whitney U test). Median survival of FISH-positive pts was 12.6 months vs. 8.1 months in FISH-negative pts (log-rank p = 0.68; HR = 0.88 [95% CI: 0.49–1.59]) and the respective figures for progression-free survival (PFS) were 7.5 vs. 4.9 months, log-rank p = 0.72; HR = 0.91 [95% CI: 0.55–1.51]. Median survival in IHC-positive vs. IHC-negative pts was 6.6 months vs. 9.2 months (log-rank p = 0.44; HR = 1.27 [95% CI: 0.69–2.36]). There was no significant difference in PFS (median of 4.8 vs. 5.3 months, respectively; log-rank p = 0.71; HR = 1.11 [95% CI: 0.64–1.92]). FISH and IHC remained insignificant in a Cox regression survival analysis. Conclusion: In this cohort of NSCLC patients treated with chemotherapy alone, EGFR FISH was associated with EGFR IHC and both features had no statistically significant influence on prognosis. [Table: see text]