Numbers of patients registered to the JSH Hematologic Disease Registry and an estimation of the annual incidence of PRCA and AA
| JSH Hematologic Disease Registry . | Year . | Estimated Incidence (95% CI) . | Total of Cohort, n (%) . | Number of Females . | Female-to-Male Ratio . | Median Age, range (yr) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 2012 . | 2013 . | 2014 . | 2015 . | 2016 . | 2017 . | 2018 . | 2019 . | |||||||
| National population (in million) | 126.0 | 125.7 | 125.4 | 125.3 | 125.3 | 125.3 | 124.2 | 123.7 | — | — | — | — | — | — |
| PRCA | 88 | 90 | 102 | 151 | 112 | 128 | 175 | 209 | 1.06 | (0.83-1.28) | 1055 (100) | 639 | 1.54 | 73 (18-99) |
| Idiopathic | 56 | 69 | 66 | 115 | 76 | 87 | 124 | 135 | — | — | 728 (69.0) | 434 | 1.48 | 75 (20-99) |
| Thymoma | 9 | 7 | 19 | 8 | 13 | 13 | 11 | 19 | — | — | 99 (9.4) | 63 | 1.75 | 67 (33-90) |
| LGL | 3 | 3 | 4 | 5 | 1 | 1 | 3 | 6 | — | — | 26 (2.5) | 13 | 1.00 | 74.5 (56-88) |
| CLL | 0 | 0 | 1 | 0 | 1 | 1 | 1 | 2 | — | — | 6 (0.6) | 4 | 2.00 | 64.5 (35-71) |
| Others | 19 | 10 | 12 | 23 | 21 | 7 | 12 | 47 | — | — | 151 (14.3) | 94 | 1.65 | 64 (18-89) |
| Not reported | 1 | 1 | 0 | 0 | 0 | 19 | 24 | 0 | — | — | 45 (4.3) | 31 | 2.21 | 72 (44-93) |
| AA | 514 | 499 | 564 | 634 | 566 | 752 | 828 | 881 | 5.24 | (4.46-6.02) | 5238 | 2882 | 1.22 | — |
| DPC study database | Year | Estimated Frequency (95% CI) | ||||||||||||
| — | — | — | 2015 | 2016 | 2017 | 2018 | 2019 | — | — | — | — | |||
| National population (in millions) | — | 125.3 | 125.3 | 125.3 | 124.2 | 123.7 | — | — | — | — | — | — | ||
| PRCA | — | — | — | 130 | 119 | 123 | 100 | 139 | 0.98 | (0.89-1.07) | — | — | — | |
| Chronic acquired PRCA | — | 47 | 57 | 52 | 46 | 80 | — | — | — | — | — | — | ||
| Transient acquired PRCA | — | 23 | 11 | 14 | 14 | 21 | — | — | — | — | — | — | ||
| Acquired PRCA, not specified | — | 60 | 51 | 57 | 40 | 38 | — | — | — | — | — | — | ||
| AA | — | — | — | 4257 | 4131 | 3887 | — | 4257 | — | — | — | — | ||
| JSH Hematologic Disease Registry . | Year . | Estimated Incidence (95% CI) . | Total of Cohort, n (%) . | Number of Females . | Female-to-Male Ratio . | Median Age, range (yr) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 2012 . | 2013 . | 2014 . | 2015 . | 2016 . | 2017 . | 2018 . | 2019 . | |||||||
| National population (in million) | 126.0 | 125.7 | 125.4 | 125.3 | 125.3 | 125.3 | 124.2 | 123.7 | — | — | — | — | — | — |
| PRCA | 88 | 90 | 102 | 151 | 112 | 128 | 175 | 209 | 1.06 | (0.83-1.28) | 1055 (100) | 639 | 1.54 | 73 (18-99) |
| Idiopathic | 56 | 69 | 66 | 115 | 76 | 87 | 124 | 135 | — | — | 728 (69.0) | 434 | 1.48 | 75 (20-99) |
| Thymoma | 9 | 7 | 19 | 8 | 13 | 13 | 11 | 19 | — | — | 99 (9.4) | 63 | 1.75 | 67 (33-90) |
| LGL | 3 | 3 | 4 | 5 | 1 | 1 | 3 | 6 | — | — | 26 (2.5) | 13 | 1.00 | 74.5 (56-88) |
| CLL | 0 | 0 | 1 | 0 | 1 | 1 | 1 | 2 | — | — | 6 (0.6) | 4 | 2.00 | 64.5 (35-71) |
| Others | 19 | 10 | 12 | 23 | 21 | 7 | 12 | 47 | — | — | 151 (14.3) | 94 | 1.65 | 64 (18-89) |
| Not reported | 1 | 1 | 0 | 0 | 0 | 19 | 24 | 0 | — | — | 45 (4.3) | 31 | 2.21 | 72 (44-93) |
| AA | 514 | 499 | 564 | 634 | 566 | 752 | 828 | 881 | 5.24 | (4.46-6.02) | 5238 | 2882 | 1.22 | — |
| DPC study database | Year | Estimated Frequency (95% CI) | ||||||||||||
| — | — | — | 2015 | 2016 | 2017 | 2018 | 2019 | — | — | — | — | |||
| National population (in millions) | — | 125.3 | 125.3 | 125.3 | 124.2 | 123.7 | — | — | — | — | — | — | ||
| PRCA | — | — | — | 130 | 119 | 123 | 100 | 139 | 0.98 | (0.89-1.07) | — | — | — | |
| Chronic acquired PRCA | — | 47 | 57 | 52 | 46 | 80 | — | — | — | — | — | — | ||
| Transient acquired PRCA | — | 23 | 11 | 14 | 14 | 21 | — | — | — | — | — | — | ||
| Acquired PRCA, not specified | — | 60 | 51 | 57 | 40 | 38 | — | — | — | — | — | — | ||
| AA | — | — | — | 4257 | 4131 | 3887 | — | 4257 | — | — | — | — | ||
The upper table depicts the number of registered patients with PRCA and AA to the JSH Hematologic Disease Registry between 2012 and 2019. A total of 1055 cases of acquired PRCA have been documented, yielding an annual incidence rate of 1.06 patients per million. The 95% CI was 0.83 to 1.28. A total of 5238 patients with AA were identified, as a reference, during the same period of observation, yielding an annual incidence rate of 5.24 per million (95% CI, 4.46-6.02). Of note, the number of patients with PRCA was 20% of those with AA in this registration. The lower table shows the number of patients with PRCA and AA in the DPC study database between 2015 and 2019. An estimated number of patients with PRCA who require hospital care is 0.98 per million (95% CI, 0.89-1.07). A total of 4175 cases with AA were documented during the same period of registration. Of note, the total number of AA in the DPC database may have been overrepresented since the category of AA in the DPC database might include those with pancytopenia other than AA.