The clinical manifestation of classic Hb H disease versus Hb H disease with Hb Constant Spring or Hb Paksé (Hb CS and PS).
| Clinical manifestation . | Hb H (--/−α) . | Hb H-Hb CS and PS (--/αCSα and --/αPSα) . |
|---|---|---|
| Number | 125 | 145 |
| Age at first transfusion (y) | 11 ± 5.5 | 1.5 ± 2.1 |
| History blood transfusion | 29% | 50% |
| Age of 1st transfusion | 12 ± 4.5 | 3 ± 2.5 |
| Growth retardation | Rare | 15% |
| Anemia | 58% | 65% |
| Jaundice | 24% | 27% |
| Hepatomegaly | 6% | 11% |
| Splenomegaly | 15% | 24% |
| Gallstones | 10% | 28% |
| Clinical manifestation . | Hb H (--/−α) . | Hb H-Hb CS and PS (--/αCSα and --/αPSα) . |
|---|---|---|
| Number | 125 | 145 |
| Age at first transfusion (y) | 11 ± 5.5 | 1.5 ± 2.1 |
| History blood transfusion | 29% | 50% |
| Age of 1st transfusion | 12 ± 4.5 | 3 ± 2.5 |
| Growth retardation | Rare | 15% |
| Anemia | 58% | 65% |
| Jaundice | 24% | 27% |
| Hepatomegaly | 6% | 11% |
| Splenomegaly | 15% | 24% |
| Gallstones | 10% | 28% |