STEM CELL TRANSPLANTATION IN PATIENTS AFFECTED BY SHWACHMAN- DIAMOND SYNDROME: EXPERT CONSENSUS AND RECOMMENDATIONS FROM THE EBMT SEVERE APLASTIC ANAEMIA WORKING PARTY

Simone Cesaro, Jean Donadieu, Marco Cipolli, Jean Hugues Dalle, Jan Styczynski, Riccardo Masetti, Brigitte Strahm, Margherita Mauro, Amal Alseraihy, Mahmoud Aljurf, Carlo Dufour, Regis Peffault de Latour
Transplantation and cellular therapy 2022 July 20
35870777
Shwachman-Diamond syndrome is a rare disorder that can develop malignant and non-malignant haematological complications. Overall, 10-20% of Shwachman-Diamond patients need hematopoietic stem cell transplantation (HSCT), but most centres have a limited experience and different approaches. The European Society for Blood and Marrow Transplantation-Severe Aplastic Anaemia Working Party promoted an expert consensus to propose recommendations regarding key issues in the management of Shwachman-Diamond patients with hematological complications. The main items identified as relevant for improving survival were: the importance of regular and structured haematologic follow-up, the potential reduction of transplant-related mortality by using reduced-intensity conditioning regimens, the limitation of total body irradiation, particularly for non-malignant severe cytopenia/bone marrow failure, the early diagnosis of clonal malignant evolution and early recognition of an indication for HSCT. Finally, the poor results of HSCT in patients with acute myeloid leukaemia, irrespective of cytoreductive chemotherapy treatment received prior to transplantation, highlights the need for innovative approaches.

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