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29 years (range, 10.2-72.6). Survival analyses demonstrat- ed a poor outcome: mortality was 6% at the age of 20, 42% at the age of 40, and 69% at the age of 60 (Figure 4A). Probability of survival after a clonal event (MDS and/or AL) was 60% by the age of 40 (Figure 4B). The 5-year sur- vival rate in patients with MDS regarding the 3 IPSS-R groups was: 30% in the high-risk group, 80% in the inter- mediate-risk group, and 100% in the low-risk group (P<0.001) (Figure 4D). Of note, severe bacterial and/or viral complications were the main causes of death in patients over the age of 40 (Figure 2C).
Myelodysplastic syndromes and AL were the main caus- es of death in 15 patients: 8 cases after chemotherapy, and 7 after HSCT. Ten patients had lethal infections: dissemi- nated mycobacterial infections in 3, bacterial infections in 3, and severe viral infections in 4 (JC virus encephalitis, onco- genic HPV, H1N1 flu, and EBV lymphoproliferative disease
post HSCT). One patient died from an aortic dissection and another from metastatic carcinoma.
Twenty-eight patients underwent HSCT for MDS or AL and/or immune deficiency. The overall survival rate of these patients was 73% after one year and 62% after five years, which then plateaued. Nine of the 28 patients died from severe infections or graft-versus-host disease. Survival after HSCT was dependent on the age at trans- plantation (Figure 4): the earlier the HSCT was performed, the better the outcome, even if the difference was not sta- tistically significant.
In cases of AL (n=18), an aggressive chemotherapy induction regimen was proposed for 16 patients, with pri- mary failure in 12 and severe infectious toxicity in 9 cases (5 cases of fungal infection). A demethylating agent was given to 3 patients and has allowed long-term disease management for MDS (n=1) and AML (n=2).
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CD
Figure 4. Survival in 79 patients. (A) Kaplan-Meier curves showing overall survival of the whole cohort. (B) Survival after a clonal event. (C) Overall survival was stud- ied after hematopoietic stem cell transplantation (HSCT) depending on the age (years) at transplantation (P>0.05). (D) Overall survival of patients with myelodys- plastic syndromes (MDS) and/or acute leukemia (AL) was plotted according to the revised International Prognostic Scoring System (IPSS-R) score at the time of the diagnosis of the malignancy. Survival significantly depends on the IPSS score (P<0.001). Confidence intervals of 95% are shaded gray. y: years.
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