Abstract
We examined SCC development of 24 FA patients, who received HSCT from HLA-matched relatives. In our BMT center, we applied low-dose CY+LFI+ATG (n:13) as conditioning regimen for FA patients between 1992 and 1999, and CY+BU+ATG (n:11) between 1999 and 2002. The aim of this study was to investigate SCC development after HSCT and examine features of the follow-up patients. The 10-year overall survival (OS) of the group with LFI+regimen was 43%, whereas the group without LFI regimen was 60%. There was a statistically significant relationship between infections (viral/bacterial) and overall survival (Fisher's Exact test P<.001). Five out of 13 long-term (>1year) surviving patients developed SCC in the HNSCC (n:4) and esophagus (n:2) region (a patient with oral SCC developed a second primary esophageal SCC). The SCC rate in our FA patients was 38%, four of the SCC patients were transplanted with irradiation used conditioning regimens, three of them had acuteGvHD (Grade II-III), only one developed chronic GvHD. The interval between HSCT and SCC diagnosis was median 13 (range 6-18) years, the age for the development of cancer was median 21 (range 15-32) years. Survival after SCC was low, median 6months (range 6-12), due to delayed SCC diagnosis, tumor progression under therapy and treatment-related toxicities of the usually reduced RT and/or CT.
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