Clinical features and prognosis of patients with myelodysplastic syndromes who were exposed to atomic bomb radiation in Nagasaki

Abstract

There is evidence that radiation exposure is a causative factor of myelodysplastic syndromes (MDS). However, little is known about whether radiation exposure is also a prognostic factor of MDS. We investigated the impact of radiation exposure on the prognosis of MDS in Nagasaki atomic bomb survivors using the International Prognostic Scoring System (IPSS) and the revised version (IPSS‐R). Subjects were 140 patients with primary MDS diagnosed between 1985 and 2011 and evaluable for IPSS, IPSS‐R, and exposure distance. Of those, 31 were exposed at <1.5 km, 35 at 1.5–2.99 km, and 74 at ≥3.0 km. By the end of March 2014, 47 patients (34%) progressed to overt leukemia and 106 (75.7%) died. By comparing with patients exposed at ≥3.0 km, those exposed at <1.5 km had significantly higher frequencies of abnormal chromosome (P = 0.02), intermediate/poor IPSS, and intermediate/poor/very poor IPSS‐R cytogenetic category (P = 0.0001, and P < 0.0001, respectively). As with de novo MDS, multivariate Cox regression analyses revealed that cytogenetic abnormalities, IPSS karyotype, and IPSS‐R cytogenetics were significantly associated with poor survival, and cumulative incidence of leukemic transformation in MDS among atomic bomb survivors, but exposure distance was not associated with any poor outcomes. These suggest that exposure to the greater dose of atomic bomb radiation is associated with developing poor cytogenetic abnormalities in MDS, which might consequently lead to overt leukemia among atomic bomb survivors.

Effect of the duration of hospice and palliative care on the quality of dying and death in patients with terminal cancer: A nationwide multicentre study

“…In addition, symptoms such as lack of appetite, tiredness, drowsiness, sense of well-being and shortness of breath generally increase slightly over time, and then begin to worsen significantly at about 3-4 weeks before death (Seow et al, 2011). Thus, we can assume that our terminally ill cancer patients who were in HPC for fewer than 21 days already had high distress due to severe symptoms and functional disability, because they had significantly poorer scores in physical and psychological comfort (Heedman & Strang, 2001;Matsuo et al, 2016). Furthermore, the performance scores of terminal patients decrease slightly over time, and then begin to worsen significantly at 4 weeks before death (Seow et al, 2011).…”

“…Furthermore, the performance scores of terminal patients decrease slightly over time, and then begin to worsen significantly at 4 weeks before death (Seow et al, 2011). Therefore, terminal patients given late referral to HPC might be more functionally impaired and have more severe symptoms upon entry into HPC (Diamond et al, 2016;Donnelly, Walsh, & Rybicki, 1995;Elsayem et al, 2004;Jenkins, Schulz, Hanson, & Bruera, 2000;Matsuo et al, 2016;Riechelmann, Krzyzanowska, O'Carroll, & Zimmermann, 2007;Stromgren et al, 2006;Walsh, Donnelly, & Rybicki, 2000). Distress from severe symptoms and impaired functionality are the most common reasons for referral to HPC (Fadul, El Osta, Dalal, Poulter, & Bruera, 2008).…”

“…Distress from severe symptoms and impaired functionality are the most common reasons for referral to HPC (Fadul, El Osta, Dalal, Poulter, & Bruera, 2008). Thus, we can assume that our terminally ill cancer patients who were in HPC for fewer than 21 days already had high distress due to severe symptoms and functional disability, because they had significantly poorer scores in physical and psychological comfort (Heedman & Strang, 2001;Matsuo et al, 2016). Professional caregivers must identify the dramatic worsening of symptoms and performance status, which often begins at 3-4 weeks before death, so the patient can be transferred to HPC at an appropriate time.…”

Chromosomal analysis of myelodysplastic syndromes among atomic bomb survivors in Nagasaki

“…We collected clinical information of MDS patients diagnosed from 1985 to 2013 registered in the Nagasaki‐City MDS database (Iwanaga et al , ; Matsuo et al , ). This database includes information from five hospitals in Nagasaki city.…”

“…Ionizing radiation induces chromosomal and genetic abnormalities, and our retrospective cohort study of Nagasaki Atomic Bomb (A‐bomb) survivors revealed that acute radiation exposure is associated with an increased risk of developing MDS, even 40–60 years after the exposure (Iwanaga et al , ). However, subsequent analyses failed to demonstrate a significant difference in survival or transformation to leukaemia with respect to distance from the hypocentre, even though a higher frequency of complex karyotypes is observed among proximally exposed cases (Iwanaga et al , ; Matsuo et al , ). This raised questions regarding the effect of A‐bomb radiation on chromosome aberrations and its clinical meaning in MDS, and prompted us to further investigate chromosomal abnormalities and their impact on survival and leukaemia transformation in MDS among survivors in more detail.…”