Hematologic Data of Family Members
| Pedigree No. . | Sex/Age . | Hb (g/dL) . | Hb F (%) . | Hb A2 (%) . | F Cells (%) . | Xmn I-Gγ . | Aγ-bp Deletion . | |
|---|---|---|---|---|---|---|---|---|
| . | . | . | . | . | Smear . | FACS . | . | . |
| II-1 | M/64 | 15.8 | 0.4 | 2.3 | 4.5 | 2.0 | +/− | −/− |
| II-2 | F/62 | 14.4 | 0.2 | 2.2 | 1.5 | 3.0 | +/− | +/− |
| II-3 | M/70 | 15.4 | 0.3 | 2.2 | 2.0 | 4.5 | +/− | −/− |
| II-4 | M/58 | 14.8 | 0.9 | 3.0 | 14 | 17.5 | +/+ | −/− |
| II-5 | F/57 | 14.5 | 0.7 | 2.8 | 3.5 | 4.0 | −/− | −/− |
| II-6 | F/48 | 14.5 | 1.3 | 3.0 | 12 | 16 | +/− | +/− |
| II-7 | M/48 | 14.9 | 0.5 | 2.5 | 11 | 9 | +/− | −−*/− |
| II-8 | F/54 | 13.1 | 0.8 | 2.3 | 12 | 12 | +/− | −−*/− |
| II-9 | M/51 | 14.9 | 0.3 | 2.5 | 1.0 | 2.0 | +/− | −/− |
| II-10 | F/50 | 13.2 | 0.3 | 2.4 | 4.0 | 4.5 | +/− | −−*/+ |
| II-11 | M/53 | 17.0 | 0.1 | 2.5 | 0.5 | 1.5 | −/− | +/− |
| III-1 | F/40 | 14.5 | 1.0 | 1.9 | 19 | 21 | +/+ | −/− |
| III-3 | M/44 | 15.8 | 0.4 | 2.1 | 5.0 | 6.0 | +/− | −/− |
| III-5 | F/16 | 14.0 | 0.4 | 2.2 | 4.5 | 6.0 | +/+ | −/− |
| III-6 | M/36 | 16.3 | 1.4 | 2.5 | 17 | 20 | +/− | −/− |
| III-7 | F/34 | 14.0 | 0.4 | 2.8 | 3.0 | 3.0 | +/− | −/− |
| III-9 | F/33 | 12.3 | 0.9 | 3.0 | 6.5 | 10 | +/− | −/− |
| III-10 | M/32 | 16.3 | 0.3 | 2.2 | 0.5 | 1.5 | −/− | +/− |
| III-11 | F/33 | 11.1 | 0.7 | 2.5 | 8.5 | 9.0 | +/− | −/− |
| III-12 | M/36 | 15.2 | 0.4 | 2.8 | 4.0 | 4.0 | +/+ | −/− |
| III-13 | F/24 | 14.1 | 3.5 | 2.8 | 47 | 44 | +/− | −−*/+ |
| III-14 | F/22 | 14.1 | 3.3 | 2.9 | 40 | 44 | +/+ | −−*/− |
| III-15 | M/17 | 14.9 | 7.1 | 2.7 | 75 | 57 | +/+ | −−*/− |
| III-16 | F/18 | 13.8 | 0.2 | 2.5 | 2.0 | 4.0 | +/+ | −−*/− |
| III-17 | M/12 | 12.4 | 0.2 | 2.4 | 2.0 | 1.5 | +/− | −/− |
| III-19 | F/28 | 12.6 | 0.2 | 2.9 | 3.0 | 3.5 | −/− | +/+ |
| III-20 | M/27 | 17.1 | 0.2 | 2.4 | 2.5 | 4.0 | +/− | −−*/+ |
| III-21 | M/25 | 15.5 | 0.1 | 2.4 | 0.5 | 0.5 | −/− | +/+ |
| IV-2 | M/11 | 13.4 | 0.9 | 2.5 | 8.0 | 8.0 | −/− | −/− |
| IV-4 | F/16 | 14.3 | 0.6 | 3.0 | 4.0 | 4.5 | −/− | +/− |
| IV-5 | F/15 | 13.5 | 0.9 | 2.5 | 8.0 | 8.0 | +/+ | −/− |
| IV-6 | M/8 | 12.9 | 1.0 | 2.6 | 6.5 | 10 | −/− | −/− |
| IV-7 | F/5 | 12.6 | 10.8 | 2.9 | 80 | 71 | +/+ | −−*/− |
| Pedigree No. . | Sex/Age . | Hb (g/dL) . | Hb F (%) . | Hb A2 (%) . | F Cells (%) . | Xmn I-Gγ . | Aγ-bp Deletion . | |
|---|---|---|---|---|---|---|---|---|
| . | . | . | . | . | Smear . | FACS . | . | . |
| II-1 | M/64 | 15.8 | 0.4 | 2.3 | 4.5 | 2.0 | +/− | −/− |
| II-2 | F/62 | 14.4 | 0.2 | 2.2 | 1.5 | 3.0 | +/− | +/− |
| II-3 | M/70 | 15.4 | 0.3 | 2.2 | 2.0 | 4.5 | +/− | −/− |
| II-4 | M/58 | 14.8 | 0.9 | 3.0 | 14 | 17.5 | +/+ | −/− |
| II-5 | F/57 | 14.5 | 0.7 | 2.8 | 3.5 | 4.0 | −/− | −/− |
| II-6 | F/48 | 14.5 | 1.3 | 3.0 | 12 | 16 | +/− | +/− |
| II-7 | M/48 | 14.9 | 0.5 | 2.5 | 11 | 9 | +/− | −−*/− |
| II-8 | F/54 | 13.1 | 0.8 | 2.3 | 12 | 12 | +/− | −−*/− |
| II-9 | M/51 | 14.9 | 0.3 | 2.5 | 1.0 | 2.0 | +/− | −/− |
| II-10 | F/50 | 13.2 | 0.3 | 2.4 | 4.0 | 4.5 | +/− | −−*/+ |
| II-11 | M/53 | 17.0 | 0.1 | 2.5 | 0.5 | 1.5 | −/− | +/− |
| III-1 | F/40 | 14.5 | 1.0 | 1.9 | 19 | 21 | +/+ | −/− |
| III-3 | M/44 | 15.8 | 0.4 | 2.1 | 5.0 | 6.0 | +/− | −/− |
| III-5 | F/16 | 14.0 | 0.4 | 2.2 | 4.5 | 6.0 | +/+ | −/− |
| III-6 | M/36 | 16.3 | 1.4 | 2.5 | 17 | 20 | +/− | −/− |
| III-7 | F/34 | 14.0 | 0.4 | 2.8 | 3.0 | 3.0 | +/− | −/− |
| III-9 | F/33 | 12.3 | 0.9 | 3.0 | 6.5 | 10 | +/− | −/− |
| III-10 | M/32 | 16.3 | 0.3 | 2.2 | 0.5 | 1.5 | −/− | +/− |
| III-11 | F/33 | 11.1 | 0.7 | 2.5 | 8.5 | 9.0 | +/− | −/− |
| III-12 | M/36 | 15.2 | 0.4 | 2.8 | 4.0 | 4.0 | +/+ | −/− |
| III-13 | F/24 | 14.1 | 3.5 | 2.8 | 47 | 44 | +/− | −−*/+ |
| III-14 | F/22 | 14.1 | 3.3 | 2.9 | 40 | 44 | +/+ | −−*/− |
| III-15 | M/17 | 14.9 | 7.1 | 2.7 | 75 | 57 | +/+ | −−*/− |
| III-16 | F/18 | 13.8 | 0.2 | 2.5 | 2.0 | 4.0 | +/+ | −−*/− |
| III-17 | M/12 | 12.4 | 0.2 | 2.4 | 2.0 | 1.5 | +/− | −/− |
| III-19 | F/28 | 12.6 | 0.2 | 2.9 | 3.0 | 3.5 | −/− | +/+ |
| III-20 | M/27 | 17.1 | 0.2 | 2.4 | 2.5 | 4.0 | +/− | −−*/+ |
| III-21 | M/25 | 15.5 | 0.1 | 2.4 | 0.5 | 0.5 | −/− | +/+ |
| IV-2 | M/11 | 13.4 | 0.9 | 2.5 | 8.0 | 8.0 | −/− | −/− |
| IV-4 | F/16 | 14.3 | 0.6 | 3.0 | 4.0 | 4.5 | −/− | +/− |
| IV-5 | F/15 | 13.5 | 0.9 | 2.5 | 8.0 | 8.0 | +/+ | −/− |
| IV-6 | M/8 | 12.9 | 1.0 | 2.6 | 6.5 | 10 | −/− | −/− |
| IV-7 | F/5 | 12.6 | 10.8 | 2.9 | 80 | 71 | +/+ | −−*/− |
Presence of the 4-bp deletion at positions −221 to −224 of the Aγ gene is noted by +.
Denotes β cluster allele carrying the γ gene triplication (γγγ).