Survey of patients with CDA I from the German CDA registry
CDA UPN . | Age, y . | Sex . | Ethnic . | Hb, g/dL . | MCV, fL . | Absolute reticulocytes, × 109/L . | Total serum bilirubin, μM . | Max ferritin, μg/L . | Morphology . | Exon . | Status . | Citation . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 026/01 | 54 | F | DE | 89.4 | 106.0 | 70.3 | 37.14 | 3010 | LM, EM | 28 | Alive | Wendt and Heimpel,2 Heimpel et al3 |
| 026/02 | 54 | F | DE | 86.3 | 108.2 | 86.5 | 37.48 | 2450 | LM, EM | 28 | Alive | Wendt and Heimpel,2 Heimpel et al3 |
| 027/01 | 48 | F | DE | 64.0 | ND | 38.0 | 22.20 | ND | LM, EM | ND | Dead | Heimpel et al3 |
| 028/01 | 56 | M | DE | 80.0 | ND | ND | 18.89 | ND | LM, EM | ND | Dead | Heimpel et al3 |
| 076/01 | 30 | M | DE | 108.0 | ND | 116.0 | 48.70 | ND | LM, EM | ND | Dead | Meuret et al15 |
| 177/01 | 48 | F | CH | 97.6 | 106.7 | 84.6 | 33.68 | 1750 | LM | 12,24 | Alive | Heimpel10 |
| 178/01 | 42 | M | CH | 88.0 | ND | 60.9 | 43.06 | 4400 | LM | 24,26 | Alive | Heimpel10 |
| 303/01 | 40 | M | CH | 131.0 | 91.7 | 71.4 | 45.05 | 134 | LM, EM | Wild type | Alive | NA |
| 406/01 | 51 | F | DE | 94.9 | 106.0 | 62.8 | 74.27 | 1560 | LM | 23,14 | Alive | NA |
| 409/01 | 50 | F | DE | 111.6 | 109.6 | 55.4 | 32.71 | 2200 | LM | 12,24,27 | Alive | NA |
| 409/04 | 50 | M | DE | 132.6 | 108.4 | 59.0 | 27.93 | 1340 | LM | 12,24,27 | Alive | NA |
| 413/01 | 43 | F | DE | 99.1 | 109.0 | 65.8 | 20.05 | 1970 | LM | ND | Dead | NA |
| 414/01 | 38 | M | DE | 108.4 | 104.9 | 43.8 | 37.23 | 3300 | LM | 14 | Alive | NA |
| 417/01 | 51 | F | DE | 90.0 | ND | 45.0 | 23.04 | 1900 | LM, EM | ND | Dead | Shalev et al14 |
| 421/01 | 34 | F | DE | 88.1 | 103.7 | 78.0 | 45.34 | 1200 | LM | 14,15 | Alive | NA |
| 446/01 | 18 | F | DE | 114.0 | 99.0 | ND | 22.20 | 460 | LM | 23,24 | Alive | Weissbach and Breidenbach16 |
| 447/01 | 6 | F | TR | 71.8 | 86.6 | 115.5 | 41.53 | 810 | LM | 26,26 | Alive | NA |
| 488/01 | 17 | M | DE | 107.0 | 100.3 | 90.4 | 46.00 | 800 | LM, EM | 14 | Alive | NA |
| 498/01 | 28 | F | DE | 84.5 | 100.5 | 71.2 | 29.52 | 960 | LM, EM | 19 | Alive | NA |
| 512/01 | 50 | M | DE | 109.4 | 99.8 | 95.6 | 69.12 | 5000 | LM | 6,14 | Alive | NA |
| 529/01 | 34 | F | YU | 100.5 | ND | ND | 46.50 | 2450 | LM | 21 | Alive | NA |
CDA UPN . | Age, y . | Sex . | Ethnic . | Hb, g/dL . | MCV, fL . | Absolute reticulocytes, × 109/L . | Total serum bilirubin, μM . | Max ferritin, μg/L . | Morphology . | Exon . | Status . | Citation . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 026/01 | 54 | F | DE | 89.4 | 106.0 | 70.3 | 37.14 | 3010 | LM, EM | 28 | Alive | Wendt and Heimpel,2 Heimpel et al3 |
| 026/02 | 54 | F | DE | 86.3 | 108.2 | 86.5 | 37.48 | 2450 | LM, EM | 28 | Alive | Wendt and Heimpel,2 Heimpel et al3 |
| 027/01 | 48 | F | DE | 64.0 | ND | 38.0 | 22.20 | ND | LM, EM | ND | Dead | Heimpel et al3 |
| 028/01 | 56 | M | DE | 80.0 | ND | ND | 18.89 | ND | LM, EM | ND | Dead | Heimpel et al3 |
| 076/01 | 30 | M | DE | 108.0 | ND | 116.0 | 48.70 | ND | LM, EM | ND | Dead | Meuret et al15 |
| 177/01 | 48 | F | CH | 97.6 | 106.7 | 84.6 | 33.68 | 1750 | LM | 12,24 | Alive | Heimpel10 |
| 178/01 | 42 | M | CH | 88.0 | ND | 60.9 | 43.06 | 4400 | LM | 24,26 | Alive | Heimpel10 |
| 303/01 | 40 | M | CH | 131.0 | 91.7 | 71.4 | 45.05 | 134 | LM, EM | Wild type | Alive | NA |
| 406/01 | 51 | F | DE | 94.9 | 106.0 | 62.8 | 74.27 | 1560 | LM | 23,14 | Alive | NA |
| 409/01 | 50 | F | DE | 111.6 | 109.6 | 55.4 | 32.71 | 2200 | LM | 12,24,27 | Alive | NA |
| 409/04 | 50 | M | DE | 132.6 | 108.4 | 59.0 | 27.93 | 1340 | LM | 12,24,27 | Alive | NA |
| 413/01 | 43 | F | DE | 99.1 | 109.0 | 65.8 | 20.05 | 1970 | LM | ND | Dead | NA |
| 414/01 | 38 | M | DE | 108.4 | 104.9 | 43.8 | 37.23 | 3300 | LM | 14 | Alive | NA |
| 417/01 | 51 | F | DE | 90.0 | ND | 45.0 | 23.04 | 1900 | LM, EM | ND | Dead | Shalev et al14 |
| 421/01 | 34 | F | DE | 88.1 | 103.7 | 78.0 | 45.34 | 1200 | LM | 14,15 | Alive | NA |
| 446/01 | 18 | F | DE | 114.0 | 99.0 | ND | 22.20 | 460 | LM | 23,24 | Alive | Weissbach and Breidenbach16 |
| 447/01 | 6 | F | TR | 71.8 | 86.6 | 115.5 | 41.53 | 810 | LM | 26,26 | Alive | NA |
| 488/01 | 17 | M | DE | 107.0 | 100.3 | 90.4 | 46.00 | 800 | LM, EM | 14 | Alive | NA |
| 498/01 | 28 | F | DE | 84.5 | 100.5 | 71.2 | 29.52 | 960 | LM, EM | 19 | Alive | NA |
| 512/01 | 50 | M | DE | 109.4 | 99.8 | 95.6 | 69.12 | 5000 | LM | 6,14 | Alive | NA |
| 529/01 | 34 | F | YU | 100.5 | ND | ND | 46.50 | 2450 | LM | 21 | Alive | NA |
Hb indicates hemoglobin concentration; MCV, mean cellular volume; DE, German; LM, light microscopy; EM, electron microscopy; ND, no data before splenectomy was available; CH, Swiss; NA, not applicable; TR, Turkish; and YU, Yugoslavian.
Values of Hb, MCV, and total serum bilirubin are means of all representative data after the age of 15 years (”Patients and methods“). For case 447/01, who was 6 years at the time of analysis, the mean of all values after 3 months is used. For ferritin, maximal values are given.