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. 2020 Jun;24(4):e13706.
doi: 10.1111/petr.13706. Epub 2020 Apr 7.

Squamous cell carcinoma development in Fanconi anemia patients who underwent hematopoietic stem cell transplantation

Sema Anak  1 , Nevin Yalman  2 , Hülya Bilgen  1 , Elif Sepet  3 , Ayhan Deviren  4 , Başak Gürtekin  5 , Fatih Tunca  6 , Bora Başaran  7
Affiliations

Squamous cell carcinoma development in Fanconi anemia patients who underwent hematopoietic stem cell transplantation

Sema Anak et al. Pediatr Transplant. 2020 Jun.

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 (>1 year) 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 6 months (range 6-12), due to delayed SCC diagnosis, tumor progression under therapy and treatment-related toxicities of the usually reduced RT and/or CT.

Keywords: Fanconi anemia; GvHD; hematopoietic stem cell transplantation; squamous cell carcinoma.

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References

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