Table 1

CBC results

PatientPlatelet count,× 109/LMPV, fLHematocrit, proportion of 1Red blood cell count, × 1012/LMean corpuscular volume, fLRDW, %
Affected males 
    III-A 76 9.5 .402 5.1 78.9 18.4 
    III-D 177 10.8 .451 5.7 79.6 19.3 
    III-S 58 9.8 .424 5.4 78.6 17.1 
    Mean 67 10.0 .426 5.4 79.1 18.3 
Obligate carrier females 
    II-B 236 8.1 .456 5.1 90.0 13.4 
    II-H 178 7.6 .384 4.7 81.3 13.7 
    IV-F 312 8.0 .373 4.8 77.1 13.9 
    IV-M 354 7.7 .335 4.2 78.9 14.0 
    Mean 227 7.9 .390 4.7 82.1 13.7 
Unaffected 
    II-C 316 8.9 .355 4.4 81.4 18.2 
    III-T 273 8.6 .354 4.4 80.4 13.7 
    IV-D 266 7.5 .466 5.7 82.3 12.9 
    IV-E 347 8.1 .458 5.5 83.9 12.7 
PatientPlatelet count,× 109/LMPV, fLHematocrit, proportion of 1Red blood cell count, × 1012/LMean corpuscular volume, fLRDW, %
Affected males 
    III-A 76 9.5 .402 5.1 78.9 18.4 
    III-D 177 10.8 .451 5.7 79.6 19.3 
    III-S 58 9.8 .424 5.4 78.6 17.1 
    Mean 67 10.0 .426 5.4 79.1 18.3 
Obligate carrier females 
    II-B 236 8.1 .456 5.1 90.0 13.4 
    II-H 178 7.6 .384 4.7 81.3 13.7 
    IV-F 312 8.0 .373 4.8 77.1 13.9 
    IV-M 354 7.7 .335 4.2 78.9 14.0 
    Mean 227 7.9 .390 4.7 82.1 13.7 
Unaffected 
    II-C 316 8.9 .355 4.4 81.4 18.2 
    III-T 273 8.6 .354 4.4 80.4 13.7 
    IV-D 266 7.5 .466 5.7 82.3 12.9 
    IV-E 347 8.1 .458 5.5 83.9 12.7 

For patient III-S, Hb A2 was 4.5% and reticulocytes were 3.5%; for patient II-H, Hb A2 was 2.6% and reticulocytes were 2.5%.

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