Table 1 Clinical and hematologic data of patients.
From: Evaluation of the main regulators of systemic iron homeostasis in pyruvate kinase deficiency
CDAII (N = 42) | PKD (N = 41) | HS (N = 50) | Reference range | P-value | |
|---|---|---|---|---|---|
Center (Milan/Naples) | 14/28 | 24/17 | 50/0 | – | < 0.001 |
Gender (M/F) | 16/26 | 25/16 | 32/18 | – | 0.03 |
Age (years) | 33 (1–73) | 26 (1–73) | 40 (0–89) | – | 0.01 |
Age < 18 years (N, %) | 14 (33) | 18 (44) | 5 (10) | – | 0.001 |
Hematochemical and biochemical data | |||||
Hb (g/dL) | 9.5 (4.7–12.3) | 9.1 (5.5–14.5) | 13.0 (7.2–17.3) | 11.0–16.0 | < 0.001 |
MCV (fL) | 89.2 (60.0–110.0) | 94.4 (62.8–130.2) | 88.3 (72.9–105.0) | 77.0–95.0 | 0.007 |
Reticulocytes (× 109/L) | 62 (7.6–223) | 156 (139–1124) | 242 (29–571) | 20–100 | < 0.001 |
Unconj. bilirubin (mg/dL) | 1.6 (0.1–11.5) | 2.6 (0.4–18.3) | 1.9 (0.5–10.8) | 0.2–0.8 | 0.04 |
LDH (U/L) | 285 (131–718) | 361 (140–3236) | 220 (144–564) | 115–211 | 0.04 |
Iron balance | |||||
Serum ferritin (ng/mL) | 344 (48–2461) | 546 (59–4490) | 337 (33–1667) | 22–275 | 0.03 |
Serum transferrin (mg/dL) | 189 (111–290) | 209 (78–419) | 233 (146–795) | 200–360 | < 0.001 |
Transferrin saturation (%) | 81.0 (13–113) | 59.7 (22–139) | 50.7 (23–129) | 15–39 | 0.03 |
Transfusion regimen, treatment, and surgical intervention | |||||
Never transfused (N, %) | 23 (57) | 10 (26) | 41 (85) | – | < 0.001 |
Transfusion (N, %) | 17 (43) | 29 (74) | 7 (15) | – | |
Transfusion needs | – | 0.03 | |||
TD (N, %) | 5 (29) | 14 (48) | 0 | – | |
TD-pre splenectomy (N, %) | 2 (12) | 4 (14) | 0 | – | |
Occasional (N, %) | 10 (59) | 9 (32) | 7 (100) | – | |
Splenectomy (N, %) | 15 (36) | 12 (29) | 7 (14) | – | 0.03 |
Chelation (N, %) | 13 (31) | 15 (37) | 0 | – | < 0.001 |
Chelation at study time (N, %) | 9 (21) | 13 (32) | 0 | – | < 0.001 |
