Table 5.
Table of results – PPV of specific diagnoses
| ICD diagnosis | Extraction | Review | Agreement | Disagreement | PPV |
|---|---|---|---|---|---|
| Congenital hemolysis | |||||
| Congenital hemolysis | 249 | 200 | 197 | 52 | 79.1 (73.5–84.0) |
| Congenital hemolysis, nonsurgical | 235 | 200 | 197 | 38 | 83.8 (78.5–88.3) |
| D55x Enzyme deficiencies | |||||
| D56x Thalassemia | |||||
| D57x Sickle cell anemia | |||||
| D582x Other hemoglobinopathies | |||||
| D580 Hereditary spherocytosis (0–1) | |||||
| D581a Hereditary elliptocytosis | |||||
| D582x Other hemoglobinopathies | |||||
| D588 Other hereditary hemolytic anemia | |||||
| D589 Hereditary hemolytic anemia | |||||
| Hemoglobinopathies | 157 | 137 | 132 | 25 | 84.1 (77.4–89.4) |
| Hemoglobinopathies, nonsurgical | 144 | 137 | 132 | 12 | 91.7 (85.9–95.6) |
| D56x Thalassemia | |||||
| D57x Sickle cell anemia | |||||
| D582x Other hemoglobinopathies | |||||
| D56 Thalassemia | 130 | 113 | 107 | 23 | 82.3 (74.7–88.4) |
| D56 Thalassemia, nonsurgical | 122 | 113 | 107 | 15 | 87.7 (80.5–93.0) |
| D56 Thalassemia | 36 | ||||
| D560 Alpha-thalassemia | 23 | ||||
| D561 Beta-thalassemia | 43 | ||||
| D563 Thalassemia minor | 5 | ||||
| D572d Sickle cell thalassemia | 6 | ||||
| D57 Sickle cell anemia | 25 | 23 | 18 | 7 | |
| D57 Sickle cell anemia | 4 | ||||
| D572 Double heterozygous sickling disorders | 2 | ||||
| D572a Hb-SC disease | 5 | ||||
| D572d Sickle cell thalassemia | 6 | ||||
| D573 Heterozygous hemoglobin S | 6 | ||||
| D580 Hereditary spherocytosis (0–1) | 72 | 58 | 51 | 21 | 70.8 (58.9–81.0) |
| D580 Hereditary spherocytosis (0–2) | 72 | 65 | 58 | 14 | 80.6 (69.5–88.9) |
| D581a Hereditary elliptocytosis | 1 | 1 | 1 | 0 | |
| D582 Other hemoglobinopathies | 2 | 7 | 1 | 1 | |
| D582 Other hemoglobinopathies | 3 | ||||
| D582e Hb-E disease | 4 | ||||
| D588 Other hereditary hemolytic anemia | 2 | 0 | 0 | 2 | |
| D589 Hereditary hemolytic anemia, unspecified | 17 | 1 | 0 | 17 | |
| Acquired | |||||
| Acquired hemolysis | 163 | 148 | 136 | 27 | 83.4 (76.8–88.8) |
| D590 AIHA, drug-induced | 2 | ||||
| D591x Other AIHA, non-drug-induced | 13 | ||||
| D592 Hemolytic nonautoimmune anemia | 2 | ||||
| D594x Other hemolytic nonautoimmune | 7 | ||||
| D595 Paroxysmal nocturnal hemoglobinuria | 4 | ||||
| D598 Other acquired hemolytic anemia | 5 | ||||
| D599 Acquired hemolytic anemia, unspecified | 3 | ||||
| D590 AIHA, drug-induced | 5 | 2 | 0 | 5 | |
| D591 AIHA, not drug-induced | 134 | 114 | 105 | 29 | 78.4 (70.4–85.0) |
| D591a Cold-agglutinin disease | 19 | ||||
| D592 Hemolytic nonautoimmune anemia | 4 | 2 | 2 | 2 | |
| D594 Other hemolytic nonautoimmune | 14 | 8 | 5 | 9 | |
| D594c Toxic hemolytic anemia | 1 | 0 | 0 | 1 | |
| D595 Paroxysmal nocturnal hemoglobinuria | 5 | 4 | 4 | 1 | |
Notes: The lowercase suffix on D572, D582, D591, and D594 refers to the specific Danish implementation of ICD-10 using lowercase letters to denote subtypes. Bold figures refer to classification lines (congital versus acquired) and major subgroups (congenital all forms, all forms of hemoglobinopathies thalassemia, sickle cell anaemia, and spherocytosis, all forms of acquired hemolysis and AIHA [not drug-induced]).
Abbreviations: ICD-10, International Classification of Diseases Tenth Revision; AIHA, autoimmune hemolytic anemia; PPV, positive predictive value; Hb-SC, sickle cell–hemoglobin C; Hb-E, hemoglobin E.