Erişkin aplastik anemi hastaları allojenik hematopoietik kök hücre nakli sonrası miks kimerik durumda remisyonda kalabilir

Öz

Amaç: Allojenik kök hücre nakli (AKHN), uygun vericisi bulunan fit ağır aplastik anemili (SAA) ve çok ağır aplastik anemili (VSAA)hastalarda küratif tedavi yöntemi olarak uygulanmaktadır. Yaş, nakil sonrası sağkalımı belirleyen en önemli faktörlerden biridir ve ilerleyen yaş ile birlikte sağkalım oranları azalmaktadır, bu nedenle uygun hastaların geciktirilmeden nakle alınması büyük önem taşımaktadır. AKHN sonrasında miks kimerizm (MK) olması nüs ya da engraftman yetersizliğinin işareti olabilir. Biz bu çalışmada, erişkin SAA ve VSAA hastalarında AKHN sonrasındaki durumunu ve takipte kimerizmin önemini incelemeyi amaçladık. Yöntemler: Merkezimizde AKHN olan 16 AA hastası bu gözlemsel çalışmaya dahil edildi. HLA durumu, hazırlama rejimi, kimerizm ve remisyon durumu kaydedildi. Bulgular: Tüm hastalarda medyan PFS 70,4 ay ve medyan OS 89,7 ay olarak izlendi. Takip süresi boyunca, 9 hasta tam kimerik iken 6 hasta MK ve 1 hastada kimerizm <%5 izlendi. Kimerizmi <%5 olan hastada engraftman kaybı izlenirken, diğer tam kimerik ve miks kimerik hastalar remisyonda izlendi. Sonuç: Minimum toksisite ile engraftmanı sağlayan optimize hazırlama rejimleri kullanılarak daha fazla hasta allo-SCT ile tedavi edilebilir. Çalışmamız, yetişkin AA hastalarının allo-SCT'den sonra, miks kimerik bir durumda bile uzun süre remisyon sağlayabildiğini göstermiştir.

Anahtar Kelimeler

• erişkin aplastik anemi
• allojenik kök hücre nakli
• kimerizm

Adult aplastic anemia patients can maintain remission after allogeneic hematopoietic stem cell transplantation in a mixed chimeric state

Öz

Aim: Allogeneic stem cell transplantation (allo-SCT) is performed as a curative treatment in young, fit, severe aplastic anemia (SAA) and very severe aplastic anemia (VSAA) patients. Age is one of the most significant factors that impact survival after transplantation. With advancing age, survival rates decrease, therefore it is crucial to perform allo-SCT without any delay in appropriate SAA and VSAA patients. Mixed chimerism (MC) in the post-allo-SCT period may be a sign of relapse or graft failure. In this study, we aimed to evaluate the outcome of allo-SCT in adult SAA and VSAA patients and the significance of chimerism monitoring in follow up. Methods: The data of 16 adult AA patients who underwent allo-SCT at our center were included in this observational study. HLA match, conditioning regimens, chimerism and remission status were recorded. Results: Median PFS was 70.4 months and median OS was 89.7 months in all patients. During the follow-up period, 9 patients remained fully chimeric whereas 6 patients had MC. The chimerism level of one patient, who had engraftment failure, was <5%. Both fully chimeric and mixed chimeric patients remained in remission. Conclusion: More AA patients can be treated with allo-SCT with more optimized conditioning regimens that provide sustained engraftment with minimum toxicity. Our study showed that adult AA patients can maintain remission for a long time after allo-SCT, even in a mixed chimeric state.

Anahtar Kelimeler

• adult aplastic anemia
• allogeneic stem cell transplantation
• chimerism

Kaynakça

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