Table 1. Clinical characteristics of children with hereditary hemolytic anemia in Korea.
| Characteristics | Membranopathies (n = 263) | Hemoglobinopathies (n = 59) | Enzymopathies (n = 23) | P value | |
|---|---|---|---|---|---|
| Age, yr | 3.0 (0–15.6) | 3.4 (0–14.9) | 0.8 (0–13.3) | 0.116 | |
| Sex, M:F | 152:111 | 39:20 | 15:8 | 0.264 | |
| Initial symptom or sign | |||||
| Skin pallor | 95 (36.1) | 19 (32.2) | 17 (73.9) | 0.014 | |
| Jaundice | 112 (42.6) | 4 (6.8) | 5 (21.7) | < 0.001 | |
| Splenomegaly | 62 (23.6) | 2 (3.4) | 3 (13) | 0.004 | |
| Hepatomegaly | 15 (5.7) | 2 (3.4) | 3 (13) | 0.559 | |
| Abdominal pain | 9 (3.4) | 1 (1.7) | 0 (0) | 0.310 | |
| Gallbladder stone | 7 (2.7) | 0 (0) | 1 (4.3) | 1.000 | |
| Growth retardation | 0 (0) | 1 (1.7) | 1 (4.3) | 0.027 | |
| Asymptomatic | 22 (8.4) | 34 (57.6) | 5 (21.7) | < 0.001 | |
| Laboratory tests | |||||
| Hemoglobin, g/dL | 9.5 (2.7–18.2) | 10.7 (4.8–17.1) | 7.5 (3.9–12.5) | < 0.001 | |
| MCV, fl | 81.1 (51–112.4) | 62 (49.5–107.6) | 94.8 (81.1–126.8) | < 0.001 | |
| MCH, pg | 28.6 (14.4–39) | 19.6 (13.6–36) | 31.9 (26.4–42.6) | < 0.001 | |
| MCHC, g/dL | 35 (28–38.8) | 31.8 (24.4–35) | 33 (30.2–36.4) | < 0.001 | |
| C-reticulocyte, % | 5.5 (0.1–22.3) | 1.2 (0.2–7.7) | 2.9 (0.5–43.5) | < 0.001 | |
| Total bilirubin, mg/dL | 2.4 (0.2–32.5) | 0.6 (0.2–32.2) | 2.6 (0.8–17.7) | < 0.001 | |
| Direct bilirubin, mg/dL | 0.6 (0.1–14.7) | 0.2 (0.1–2.9) | 0.7 (0.2–1.6) | < 0.001 | |
| LDH, IU/L | 524 (181–1,616) | 385 (159–1,623) | 856 (207–11,630) | 0.001 | |
| Haptoglobin, mg/dL | 7.8 (1.1–272) | 82 (7–349) | 8.6 (1–20) | < 0.001 | |
Data are presented as median (range) or number (%).
MCV = mean corpuscular volume, MCH = mean corpuscular hemoglobin, MCHC = mean corpuscular hemoglobin concentration, C-reticulocyte = corrected reticulocyte, LDH = lactate dehydrogenase, M = male, F = female.