AI Article Synopsis

  • A rare case of a 33-year-old man developed pro-B-acute lymphoblastic leukemia (ALL) from myelodysplastic syndrome (MDS) with identified genetic mutation (S1231F) that may influence disease progression but isn't a negative prognostic marker.
  • An analysis of 30 MDS patients showed the majority transformed to ALL within a median of 5.5 months, predominantly male with a median age of 56, and types included MDS-excess blasts and MDS-single lineage dysplasia.
  • The study revealed a 75% complete remission rate with ALL-directed chemotherapy, yet the transformation was linked to a concerning early death rate of 20% among these patients.

Article Abstract

The development of acute lymphoblastic leukemia (ALL) from myelodysplastic syndrome (MDS) is a very rare event. The current report presents a rare case of a 33-year-old man who was diagnosed with MDS with multiple-lineage dysplasia (MDS-MLD) that transformed into pro-B-ALL. A missense mutation (S1231F) of the additional sex combs like 1, transcriptional regulator gene was identified, which may have a substantial role in the progression, however does not act as an unfavorable prognostic marker. The patient died during induction chemotherapy. The present study further conducted an analysis on 30 patients to determine progression to ALL. Patients were predominantly male (76.7%, 23/30) with a median age of 56 years (3-90 years). The median time to transformation was 5.5 months (2-50 months). The most common type of MDS with ALL transformation comprised of MDS-excess blasts (MDS-EB; 40%, 12/30), MDS with single-lineage dysplasia (MDS-SLD; 30%, 9/30) and MDS with ring sideroblasts (MDS-RS; 16.7%, 5/30). The majority of the patients transformed to B-cell (66.7%, 16/24) followed by T-cell (33.3%, 8/24) ALL. From the 25 cases where data was available, the complete remission rate was 75% (15/20) with ALL-directed chemotherapy and the median remission duration was 15 months (range 4.5 to 51 months). However, the results indicated that ALL following MDS is characterized by a high rate of early death (20%, 5/25).

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5958712PMC
http://dx.doi.org/10.3892/ol.2018.8546DOI Listing

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