Cohort of patients with PRCA
| . | Changes in megakaryocyte only . | Changes in thrombocytosis only . | Changes in both . | Changes in megakaryocyte and/or thrombocytosis . | Neither (PRCA in the absence of thrombocytosis or megakaryocyte changes) . | Total . |
|---|---|---|---|---|---|---|
| Number of patients | 16 | 4 | 7 | 27 | 63 | 90 |
| Mean age at diagnosis, y | 57.6 ± 8.3 (33.8-82) | 68 ± 16.1 (45.3-84.3) | 53.5 ± 11.2 (28-73.9) | 58.1 ± 6.2 (28-84.3) | 56.7 ± 5.1 (0-85) | 57.1 ± 4 (0-85) |
| Patients with BM megakaryocyte changes | ||||||
| Dysplasia | 1 | 0 | 0 | 1 | 0 | 1 |
| Hyperlobation | 3 | 0 | 1 | 4 | 0 | 4 |
| Hyperplasia | 13 | 0 | 7 | 20 | 0 | 20 |
| Total with any changes | 16 | 0 | 7 | 23 | 0 | 23 |
| Mean hemoglobin at diagnosis, g/dL | 7.6 ± 0.9 (3.5-9.6) | 6.4 ± 1.3 (4.5-7.4) | 8.3 ± 1.6 (5.1-12.4) | 7.6 ± 0.7 (3.5-12.4) | 7.7 ± 0.6 (2.6-11.7) | 7.6 ± 0.5 (2.6-12.4) |
| Mean platelet count, × 109/L | 232.7 ± 70.3 (9-440) | 550.8 ± 96.6 (476-695) | 564.7 ± 59.2 (472-693) | 389 ± 80.4 (9-695) | 254.2 ± 26.7 (45-432) | 299.1 ± 35.2 (9-695) |
| Mean ferritin at diagnosis, μg/L | 2309.4 ± 1750.2 (648.4-6579) | 2254.9 ± 2350.7 (434.1-5505) | 705 ± 310.1 (315-1 569) | 1 635.9 ± 861.5 (315-6 579) | 2 021 ± 583.6 (50.1-7 597) | 1 899.8 ± 481 (50.1-7 597) |
| Mean WBC at diagnosis, 1 000 WBC per μL | 8.1 ± 2.7 (3.9-18.8) | 8.3 ± 0.7 (7.8-9.4) | 8 ± 1.8 (3.3-10.3) | 8.1 ± 1.5 (3.3-18.8) | 5.6 ± 0.7 (2-12.6) | 6.4 ± 0.7 (2-18.8) |
| Female (% of total) | 6 (37.5%) | 2 (50%) | 6 (85.7%) | 14 (51.9%) | 29 (46.0%) | 43 (47.8%) |
| Mean BM cellularity, % | 55.3 ± 10.9 (25-90) | 53.8 ± 26.9 (40-95) | 73.6 ± 8.2 (50-80) | 60 ± 8.2 (25-95) | 39.6 ± 5.4 (5-90) | 1 973.2 ± 745.8 (46.6-11 560) |
| Next generation sequencing findings | • STAT3 p.S614RC 8 • IDH1, SETBP1 | • SF3B1 p.K666N 18.21% • BCOR p.1252_1253del 9% | • c.490G>T p.G164C 43.2%, c.1292C>T p.P431L 41.7%, c.6460G>A p.D2154N 41.4%, c.3974A>G p.K1325R 40.6%, c.588_589insACCCGC p.P196_P197insTR 13.0% • NF-kappaB2, JAK2, TYK 2 • c.490G>T p.G164C 43.2%, c.1292C>T p.P431L 41.7%, c.6460G>A p.D2154N 41.4%, c.3974A>G p.K1325R 40.6%, c.588_589insACCCGC p.P196_P197insTR 13.0% | • SPTB, SPTA1, EPB42 • TET2 p.N275lfs 4.3% • RPS19, HFE C282Y, H63D • PB, ASXL1, U2AF1 • ASXL1, JAK2, STAT3, U2AF1 VUS • STAT3 p.D661Y VAF 12.7% • ASXL1 p.K912Q c.2734A>C VAF 50.3% and PTPN11 p.K131R c.392A>G 51.1% VAF N6471 mutation in STAT3 gene | ||
| Mean absolute reticulocyte count at diagnosis, reticulocytes per μL | 2.924 ± 3.162 (0.01-10) | 0.013 ± 0.004 (0.011-0.015) | 3.685 ± 3.322 (0.007-8.9) | 2.84 ± 1.987 (0.007-10) | 1.45 ± 0.884 (0-7.1) | 1.894 ± 0.881 (0-10) |
| Mean reticulocyte percent at diagnosis, % | 0.684 ± 0.647 (0.3-2) | 0.543 ± 0.283 (0.3-0.8) | 0.263 ± 0.084 (0.04-0.4) | 0.459 ± 0.231 (0.04-2) | 0.5 ± 0.191 (0.1-2.1) | 0.487 ± 0.149 (0.04-2.1) |
| Mean erythropoietin level, mU/mL | 748.6 ± 532.1 (46.6-1646) | 2012 (n = 1) | 2 553.9 ± 3 290 (72.4-10 815) | 1 679 ±1 542.6 (46.6-10 815) | 2 105 ± 845.3 (91-11 560) | 1 973.2 ± 745.8 (46.6-11 560) |
| Parvovirus B19 | 0 | 2 | 1 | 3 | 3 | 6 |
| Chronic lymphocytic leukemia | 0 | 1 | 0 | 1 | 1 | 2 |
| Large granular lymphocyte leukemia | 4 | 2 | 4 | 10 | 15 | 25 |
| . | Changes in megakaryocyte only . | Changes in thrombocytosis only . | Changes in both . | Changes in megakaryocyte and/or thrombocytosis . | Neither (PRCA in the absence of thrombocytosis or megakaryocyte changes) . | Total . |
|---|---|---|---|---|---|---|
| Number of patients | 16 | 4 | 7 | 27 | 63 | 90 |
| Mean age at diagnosis, y | 57.6 ± 8.3 (33.8-82) | 68 ± 16.1 (45.3-84.3) | 53.5 ± 11.2 (28-73.9) | 58.1 ± 6.2 (28-84.3) | 56.7 ± 5.1 (0-85) | 57.1 ± 4 (0-85) |
| Patients with BM megakaryocyte changes | ||||||
| Dysplasia | 1 | 0 | 0 | 1 | 0 | 1 |
| Hyperlobation | 3 | 0 | 1 | 4 | 0 | 4 |
| Hyperplasia | 13 | 0 | 7 | 20 | 0 | 20 |
| Total with any changes | 16 | 0 | 7 | 23 | 0 | 23 |
| Mean hemoglobin at diagnosis, g/dL | 7.6 ± 0.9 (3.5-9.6) | 6.4 ± 1.3 (4.5-7.4) | 8.3 ± 1.6 (5.1-12.4) | 7.6 ± 0.7 (3.5-12.4) | 7.7 ± 0.6 (2.6-11.7) | 7.6 ± 0.5 (2.6-12.4) |
| Mean platelet count, × 109/L | 232.7 ± 70.3 (9-440) | 550.8 ± 96.6 (476-695) | 564.7 ± 59.2 (472-693) | 389 ± 80.4 (9-695) | 254.2 ± 26.7 (45-432) | 299.1 ± 35.2 (9-695) |
| Mean ferritin at diagnosis, μg/L | 2309.4 ± 1750.2 (648.4-6579) | 2254.9 ± 2350.7 (434.1-5505) | 705 ± 310.1 (315-1 569) | 1 635.9 ± 861.5 (315-6 579) | 2 021 ± 583.6 (50.1-7 597) | 1 899.8 ± 481 (50.1-7 597) |
| Mean WBC at diagnosis, 1 000 WBC per μL | 8.1 ± 2.7 (3.9-18.8) | 8.3 ± 0.7 (7.8-9.4) | 8 ± 1.8 (3.3-10.3) | 8.1 ± 1.5 (3.3-18.8) | 5.6 ± 0.7 (2-12.6) | 6.4 ± 0.7 (2-18.8) |
| Female (% of total) | 6 (37.5%) | 2 (50%) | 6 (85.7%) | 14 (51.9%) | 29 (46.0%) | 43 (47.8%) |
| Mean BM cellularity, % | 55.3 ± 10.9 (25-90) | 53.8 ± 26.9 (40-95) | 73.6 ± 8.2 (50-80) | 60 ± 8.2 (25-95) | 39.6 ± 5.4 (5-90) | 1 973.2 ± 745.8 (46.6-11 560) |
| Next generation sequencing findings | • STAT3 p.S614RC 8 • IDH1, SETBP1 | • SF3B1 p.K666N 18.21% • BCOR p.1252_1253del 9% | • c.490G>T p.G164C 43.2%, c.1292C>T p.P431L 41.7%, c.6460G>A p.D2154N 41.4%, c.3974A>G p.K1325R 40.6%, c.588_589insACCCGC p.P196_P197insTR 13.0% • NF-kappaB2, JAK2, TYK 2 • c.490G>T p.G164C 43.2%, c.1292C>T p.P431L 41.7%, c.6460G>A p.D2154N 41.4%, c.3974A>G p.K1325R 40.6%, c.588_589insACCCGC p.P196_P197insTR 13.0% | • SPTB, SPTA1, EPB42 • TET2 p.N275lfs 4.3% • RPS19, HFE C282Y, H63D • PB, ASXL1, U2AF1 • ASXL1, JAK2, STAT3, U2AF1 VUS • STAT3 p.D661Y VAF 12.7% • ASXL1 p.K912Q c.2734A>C VAF 50.3% and PTPN11 p.K131R c.392A>G 51.1% VAF N6471 mutation in STAT3 gene | ||
| Mean absolute reticulocyte count at diagnosis, reticulocytes per μL | 2.924 ± 3.162 (0.01-10) | 0.013 ± 0.004 (0.011-0.015) | 3.685 ± 3.322 (0.007-8.9) | 2.84 ± 1.987 (0.007-10) | 1.45 ± 0.884 (0-7.1) | 1.894 ± 0.881 (0-10) |
| Mean reticulocyte percent at diagnosis, % | 0.684 ± 0.647 (0.3-2) | 0.543 ± 0.283 (0.3-0.8) | 0.263 ± 0.084 (0.04-0.4) | 0.459 ± 0.231 (0.04-2) | 0.5 ± 0.191 (0.1-2.1) | 0.487 ± 0.149 (0.04-2.1) |
| Mean erythropoietin level, mU/mL | 748.6 ± 532.1 (46.6-1646) | 2012 (n = 1) | 2 553.9 ± 3 290 (72.4-10 815) | 1 679 ±1 542.6 (46.6-10 815) | 2 105 ± 845.3 (91-11 560) | 1 973.2 ± 745.8 (46.6-11 560) |
| Parvovirus B19 | 0 | 2 | 1 | 3 | 3 | 6 |
| Chronic lymphocytic leukemia | 0 | 1 | 0 | 1 | 1 | 2 |
| Large granular lymphocyte leukemia | 4 | 2 | 4 | 10 | 15 | 25 |
BM, bone marrow; WBC, white blood cell.