Characteristics and actuarial risk of clonal or malignant myeloid transformation (CMMT) among patients with IBMFS. (A) Actuarial risk of CMMT was estimated by Kaplan-Meier analysis. Censoring was performed at last follow-up or at the time of hematopoietic stem cell transplantation for patients who had undergone such a procedure. The figure represents risk with 95% confidence intervals. (B) Risk of progression to more advanced transformational stage among patients with CMMT. The figure represents risk with 95% confidence intervals. (C) Overall survival analysis of patients with and without CMMT up to the age of 18 years. (D-I) Actuarial risk of CMMT among the common categories of inherited bone marrow failure syndromes: Fanconi anemia (FA), Shwachman-Diamond syndrome (SDS), dyskeratosis congenital (DC), Kostmann/severe congenital neutropenia (K/SCN), unclassified-IBMFS (UC-IBMFS), and comparison between FA, SDS and DBA. The figures represent risk with 95% confidence intervals.