Cause-specific mortality during 1977 to 1999 in males in the United Kingdom with hemophilia A or B who were not infected with HIV compared with national mortality
| Certified underlying cause of death (ICD9 code) . | Severe hemophilia . | Moderate/mild hemophilia . | Total . | |||||
|---|---|---|---|---|---|---|---|---|
| Observed deaths . | Expected deaths . | O/E* . | Observed deaths . | Expected deaths . | O/E* . | O/E* . | 95% CI . | |
| A Causes that were significantly increased in the hemophilia population | ||||||||
| Coagulation defects etc (280-289) | 14 | 0.25 | 55.56§ | 9 | 1.33 | 6.77§ | 14.55§ | 9.22-21.83 |
| Intracranial hemorrhage (ICH, 430-432)† | 62 | 1.58 | 39.29§ | 64 | 6.89 | 9.29§ | 14.88§ | 12.40-17.72 |
| Other or unspecified hemorrhage (OH, 459.0, 578) | 27 | 0.08 | 341.77§ | 15 | 0.54 | 27.73§ | 67.74§ | 48.84-91.57 |
| Injury excl suicide and poisoning (E800-999 excl.E850-869, E950-959, E980-989) | 17 | 6.00 | 2.84§ | 25 | 16.34 | 1.53 | 1.88§ | 1.36-2.54 |
| Hepatitis and liver disease (070, 570-573) | 12 | 1.02 | 11.72§ | 35 | 4.70 | 7.44§ | 8.21§ | 6.03-10.91 |
| Liver cancer (155) | 10 | 0.30 | 32.89§ | 17 | 1.70 | 10.03§ | 13.51§ | 8.90-19.65 |
| Ill-defined cerebrovascular disease (IDCD, 436-438) | 20 | 3.48 | 5.76§ | 28 | 23.69 | 1.18 | 1.77§ | 1.30-2.34 |
| Digestive system excl. liver and hemorrhage (520-579 excl. 570-573, 578) | 4 | 1.60 | 2.49 | 16 | 8.90 | 1.80* | 1.90* | 1.16-2.94 |
| Hodgkin disease (201)‡ | 1 | 0.20 | 4.98 | 3 | 0.61 | 4.94* | 4.95* | 1.35-12.67 |
| All causes in category A | 167 | 14.51 | 11.51§ | 212 | 64.69 | 3.28§ | 4.78§ | 4.32-5.29 |
| B Ischemic heart disease (IHD, 410-414) | 16 | 25.84 | 0.62 | 88 | 140.69 | 0.63§ | 0.62§ | 0.51-0.76 |
| C Other causes | ||||||||
| Infections excl. hepatitis (001-139,279 excl. 070) | 3 | 0.83 | 3.62 | 1 | 3.27 | 0.31 | 0.98 | 0.27,2.50 |
| Non-Hodgkin lymphoma (200,202) | 0 | 0.74 | 0.00 | 3 | 3.65 | 0.82 | 0.68 | 0.14-2.00 |
| Other neoplasms (140-239 excl 155, 200-202) | 15 | 23.23 | 0.65 | 122 | 128.34 | 0.95 | 0.90 | 0.76-1.07 |
| Endocrine disorders (240-278) | 3 | 1.15 | 2.60 | 3 | 6.04 | 0.50 | 0.83 | 0.31-1.82 |
| Mental disorders (290-319) | 0 | 0.76 | 0.00 | 2 | 4.38 | 0.46 | 0.39 | 0.05-1.41 |
| Nervous system disorders (320-389) | 1 | 1.89 | 0.53 | 6 | 8.38 | 0.72 | 0.68 | 0.27-1.40 |
| Ischemic stroke (IS, 433-435) | 1 | 0.96 | 1.04 | 3 | 5.38 | 0.56 | 0.63 | 0.17-1.62 |
| Circulatory excl ICH, OH, IDCD, IHD & IS (390-459 excl 410-414, 430-438, 459.0) | 8 | 5.74 | 1.39 | 36 | 32.53 | 1.11 | 1.15 | 0.84-1.54 |
| Pneumonia (480-486) | 7 | 3.21 | 2.18 | 21 | 18.87 | 1.11 | 1.27 | 0.84-1.83 |
| Other respiratory (460-519 excl. 480-486) | 7 | 5.27 | 1.33 | 23 | 31.80 | 0.72 | 0.81 | 0.55-1.16 |
| Genitourinary diseases (580-629) | 3 | 0.75 | 4.00 | 7 | 4.30 | 1.63 | 1.98 | 0.95-3.64 |
| Musculoskeletal & connective tissue (710-739) | 2 | 0.24 | 8.20 | 1 | 1.42 | 0.71 | 1.81 | 0.37-5.28 |
| Other diseases (630-709, 740-799) | 2 | 2.32 | 0.86 | 8 | 4.58 | 1.75 | 1.45 | 0.70-2.67 |
| Suicide and poisoning (E850-869, E950-959, E980-989) | 7 | 3.88 | 1.81 | 15 | 12.51 | 1.20 | 1.34 | 0.84-2.03 |
| All causes in category C | 59 | 50.97 | 1.16 | 251 | 265.43 | 0.95 | 0.98 | 0.87-1.10 |
| D Unknown cause | 4 | — | — | 9 | — | — | — | |
| All causes | 246 | 91.33 | 2.69§ | 560 | 470.81 | 1.19§ | 1.43§ | 1.34-1.54 |
| Certified underlying cause of death (ICD9 code) . | Severe hemophilia . | Moderate/mild hemophilia . | Total . | |||||
|---|---|---|---|---|---|---|---|---|
| Observed deaths . | Expected deaths . | O/E* . | Observed deaths . | Expected deaths . | O/E* . | O/E* . | 95% CI . | |
| A Causes that were significantly increased in the hemophilia population | ||||||||
| Coagulation defects etc (280-289) | 14 | 0.25 | 55.56§ | 9 | 1.33 | 6.77§ | 14.55§ | 9.22-21.83 |
| Intracranial hemorrhage (ICH, 430-432)† | 62 | 1.58 | 39.29§ | 64 | 6.89 | 9.29§ | 14.88§ | 12.40-17.72 |
| Other or unspecified hemorrhage (OH, 459.0, 578) | 27 | 0.08 | 341.77§ | 15 | 0.54 | 27.73§ | 67.74§ | 48.84-91.57 |
| Injury excl suicide and poisoning (E800-999 excl.E850-869, E950-959, E980-989) | 17 | 6.00 | 2.84§ | 25 | 16.34 | 1.53 | 1.88§ | 1.36-2.54 |
| Hepatitis and liver disease (070, 570-573) | 12 | 1.02 | 11.72§ | 35 | 4.70 | 7.44§ | 8.21§ | 6.03-10.91 |
| Liver cancer (155) | 10 | 0.30 | 32.89§ | 17 | 1.70 | 10.03§ | 13.51§ | 8.90-19.65 |
| Ill-defined cerebrovascular disease (IDCD, 436-438) | 20 | 3.48 | 5.76§ | 28 | 23.69 | 1.18 | 1.77§ | 1.30-2.34 |
| Digestive system excl. liver and hemorrhage (520-579 excl. 570-573, 578) | 4 | 1.60 | 2.49 | 16 | 8.90 | 1.80* | 1.90* | 1.16-2.94 |
| Hodgkin disease (201)‡ | 1 | 0.20 | 4.98 | 3 | 0.61 | 4.94* | 4.95* | 1.35-12.67 |
| All causes in category A | 167 | 14.51 | 11.51§ | 212 | 64.69 | 3.28§ | 4.78§ | 4.32-5.29 |
| B Ischemic heart disease (IHD, 410-414) | 16 | 25.84 | 0.62 | 88 | 140.69 | 0.63§ | 0.62§ | 0.51-0.76 |
| C Other causes | ||||||||
| Infections excl. hepatitis (001-139,279 excl. 070) | 3 | 0.83 | 3.62 | 1 | 3.27 | 0.31 | 0.98 | 0.27,2.50 |
| Non-Hodgkin lymphoma (200,202) | 0 | 0.74 | 0.00 | 3 | 3.65 | 0.82 | 0.68 | 0.14-2.00 |
| Other neoplasms (140-239 excl 155, 200-202) | 15 | 23.23 | 0.65 | 122 | 128.34 | 0.95 | 0.90 | 0.76-1.07 |
| Endocrine disorders (240-278) | 3 | 1.15 | 2.60 | 3 | 6.04 | 0.50 | 0.83 | 0.31-1.82 |
| Mental disorders (290-319) | 0 | 0.76 | 0.00 | 2 | 4.38 | 0.46 | 0.39 | 0.05-1.41 |
| Nervous system disorders (320-389) | 1 | 1.89 | 0.53 | 6 | 8.38 | 0.72 | 0.68 | 0.27-1.40 |
| Ischemic stroke (IS, 433-435) | 1 | 0.96 | 1.04 | 3 | 5.38 | 0.56 | 0.63 | 0.17-1.62 |
| Circulatory excl ICH, OH, IDCD, IHD & IS (390-459 excl 410-414, 430-438, 459.0) | 8 | 5.74 | 1.39 | 36 | 32.53 | 1.11 | 1.15 | 0.84-1.54 |
| Pneumonia (480-486) | 7 | 3.21 | 2.18 | 21 | 18.87 | 1.11 | 1.27 | 0.84-1.83 |
| Other respiratory (460-519 excl. 480-486) | 7 | 5.27 | 1.33 | 23 | 31.80 | 0.72 | 0.81 | 0.55-1.16 |
| Genitourinary diseases (580-629) | 3 | 0.75 | 4.00 | 7 | 4.30 | 1.63 | 1.98 | 0.95-3.64 |
| Musculoskeletal & connective tissue (710-739) | 2 | 0.24 | 8.20 | 1 | 1.42 | 0.71 | 1.81 | 0.37-5.28 |
| Other diseases (630-709, 740-799) | 2 | 2.32 | 0.86 | 8 | 4.58 | 1.75 | 1.45 | 0.70-2.67 |
| Suicide and poisoning (E850-869, E950-959, E980-989) | 7 | 3.88 | 1.81 | 15 | 12.51 | 1.20 | 1.34 | 0.84-2.03 |
| All causes in category C | 59 | 50.97 | 1.16 | 251 | 265.43 | 0.95 | 0.98 | 0.87-1.10 |
| D Unknown cause | 4 | — | — | 9 | — | — | — | |
| All causes | 246 | 91.33 | 2.69§ | 560 | 470.81 | 1.19§ | 1.43§ | 1.34-1.54 |
HIV-positive patients were excluded from 1985 or date of seroconversion, if later.
—indicates not applicable.
O/E (observed deaths/expected deaths) is the multiplicative factor by which the age- and calendar year–specific mortality rate in the hemophilia population differs from the corresponding rate in the general population. It is sometimes known as the standardized mortality ratio.
In children younger than 5 years, mortality from intracranial hemorrhage was as follows: severe hemophilia O: 8, E: 0.005, O/E: 1590.43§; moderate/mild hemophilia O: 2, E: 0.008, O/E: 263.16§; Total O/E: 775.19§ (95% CI: 371.74-1425.61).
Two of the 4 deaths from Hodgkin disease were in individuals who had tested negative for HIV (in 1984 and 1986); for one there was no record of any treatment with a blood product, and for one the diagnosis of hemophilia was made only after the diagnosis of Hodgkin disease.