Inherited Hemophilia—A Multidimensional Chronic Disease That Requires a Multidisciplinary Approach
Abstract
:1. Introduction
2. Materials and Methods
2.1. Study Population
2.2. Measuring Tools
2.3. Statistical Analysis
3. Results
3.1. Demographic and Clinical Characteristics
3.2. Articular Damage
3.2.1. Distribution of Severity of Articular Damage
3.2.2. Correlation Between Form of Hemophilia and Joint Damage
- -
- A total of 53.6% of patients associated severe hemophilia with severe damage to the right knee, while in patients with mild hemophilia, damage to the right knee was absent in all patients (p = 0.028).
- -
- In the left knee, severe hemophilia was associated with severe damage in 39.3% of patients, while in patients with mild hemophilia, damage to the left knee was absent in all patients (p = 0.035).
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- A total of 32.1% of patients associated severe hemophilia with severe damage to the right ankle, while in patients with mild hemophilia, damage to the right ankle was absent in all patients, and in those with moderate hemophilia, damage to the right knee was mild in all patients (p = 0.001).
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- A total of 25% of patients associated severe hemophilia with severe damage to the left knee, while in patients with mild hemophilia, damage to the left knee was absent in all patients, and in those with moderate hemophilia, damage to the left knee was mild in all patients (p = 0.001).
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- A total of 25% of patients associated moderate hemophilia and 14.3% associated severe hemophilia with severe damage to the right hip (p = 0.709).
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- A total of 25% of patients associated moderate hemophilia and 14.3% associated severe hemophilia with severe damage to the left hip (p = 0.605).
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- A total of 14.3% of patients associated severe hemophilia with severe damage to the right wrist, while mild hemophilia was not associated with damage to the right wrist (p = 0.243).
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- Severe hemophilia was associated with mild and moderate left wrist damage in 42.8% of patients, while in patients with mild hemophilia, left wrist damage was absent in all patients (p = 0.303).
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- A total of 25% of patients associated severe hemophilia with severe damage to the right elbow, while patients with mild hemophilia did not show damage to the right elbow (p = 0.007).
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- In the left elbow, severe hemophilia was associated with moderate and severe damage in 53.5% of patients, while in patients with mild hemophilia, damage to the left fist was absent in all patients (p = 0.029).
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- All patients with mild hemophilia, 75% of patients with moderate hemophilia, and 60.7% of patients with severe hemophilia did not have damage to the right shoulder (p = 0.508).
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- All patients with mild hemophilia, 75% of patients with moderate hemophilia, and 75% of patients with severe hemophilia did not have damage to the left shoulder (p = 0.529).
3.2.3. Correlation Between Treatment and Joint Damage
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- A total of 57.1% of patients with continuous prophylaxis did not have right knee damage, while 72% of patients with short-term replacement had severe right knee damage (p = 0.001) (Table 5).
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- A total of 85.7% of patients with continuous prophylaxis had no left knee damage, while 48% of short-term substitution patients had severe left knee damage (p = 0.001) (Table 5).
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- A total of 42.9% of patients with continuous prophylaxis had mild right ankle involvement, while 36% of short-term substitution patients had severe right ankle involvement (p = 0.05) (Table 5).
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- A total of 76.7% of patients with continuous prophylaxis and 83.3% of patients with short-term substitution associated mild damage to the left ankle (p = 0.019) (Table 5).
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- All patients with continuous prophylaxis and 64% of those with short-term substitution did not have a correlation with right hip damage (p = 0.148) (Table 5).
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- All patients with continuous prophylaxis and 56% of those with short-term substitution did not associate damage to the left hip (p = 0.076) (Table 5).
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- All patients with continuous prophylaxis did not present damage to the right hip, while 36% of those with short-term substitution are associated with moderate-severe damage to the right hip (p = 0.014) (Table 5).
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- All patients with continuous prophylaxis did not have damage to the right wrist, while 36% of those with short-term substitution had an association with moderate–severe damage to the right wrist (p = 0.014) (Table 5).
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- All patients with continuous prophylaxis did not have damage to the left wrist, while 36% of those with short-term substitution had an association with moderate–severe damage to the left wrist (p = 0.014) (Table 5).
- -
- A total of 28% of patients with short-term substitution associated severe damage to the right elbow, while patients with continuous prophylaxis did not present damage to the right elbow (p = 0.025) (Table 5).
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- A total of 85.7% of patients with continuous prophylaxis did not have damage to the left elbow, while 60% of the patients with short-term substitution were associated with moderate–severe damage to the left elbow (p = 0.002) (Table 5).
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- A total of 85.7% of patients with continuous prophylaxis and 56% of patients with short-term substitution did not have an association with right shoulder damage (p = 0.147) (Table 5).
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- All of the patients with continuous prophylaxis and 68% of those with short-term substitution did not have an association with damage to the left shoulder (p = 0.098) (Table 5).
3.3. Comorbidities and Hemophilia
3.3.1. Correlation Between Form of Hemophilia and HCV Infection
3.3.2. Correlation Between Joint Damage and HCV Infection
3.3.3. Correlation Between Treatment and HCV Infection
4. Discussion
4.1. The Clinical Implications of the Study
4.2. Study Limitations
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Characteristics | n | % |
---|---|---|
Gender | ||
Male
Female |
34
2 |
94.4
5.6 |
Age | ||
<45 years old
≥45 years old |
17
19 |
47.2
52.8 |
Urban vs. rural | ||
Urban
Rural |
15
21 |
41.7
58.3 |
Type of hemophilia | ||
Type A
Type B |
30
6 |
83.3
16.7 |
Forms of hemophilia | ||
Mild
Moderate Severe |
4
4 28 |
11.1
11.1 77.8 |
Treatment | ||
On demand
Continuous prophylaxis Intermittent prophylaxis |
4
7 25 |
11.1
19.5 69.4 |
Joint | Absent | Mild | Moderate | Severe | p |
---|---|---|---|---|---|
Right knee | 10 (27.8%) | 2 (5.6%) | 6 (16.7%) | 18 (50.0%) | 0.001 |
Left knee | 12 (33.3%) | 4 (11.1%) | 8 (22.2%) | 12 (33.3%) | |
Right ankle | 7 (19.4%) | 10 (27.8%) | 10 (27.8%) | 9 (25.0%) | 0.002 |
Left ankle | 7 (19.4%) | 16 (44.4%) | 6 (16.7%) | 7 (19.4%) | |
Right hip | 27 (75,0%) | 3 (8.3%) | 1 (2.8%) | 5 (13.9%) | 0.601 |
Left hip | 25 (69.4%) | 4 (11.1%) | 2 (5.6%) | 5 (13.9%) | |
Right wrist | 21 (58.3%) | 6 (16.7%) | 5 (13.9%) | 4 (11.1%) | 0.136 |
Left wrist | 21 (58.3%) | 6 (16.7%) | 7 (19.4%) | 2 (5.6%) | |
Right elbow | 13 (36.1%) | 6 (16.7%) | 10 (27.8%) | 7 (19.4%) | 0.809 |
Left elbow | 14 (38.9%) | 7 (19.4%) | 9 (25.0%) | 6 (16.7%) | |
Right shoulder | 24 (66.7%) | 3 (8.3%) | 6 (16.7%) | 3 (8.3%) | 0.296 |
Left shoulder | 28 (77.8%) | 3 (8.3%) | 5 (13.9%) | - |
Joint Score | Study | At Least 3 Joints Affected | ||
---|---|---|---|---|
No | % | n | % | |
Absent | 4 | 11.1 | ||
Mild | 5 | 13.9 | 1 | 20.0 |
Moderate | 4 | 11.1 | 1 | 25.0 |
Severe | 23 | 63.9 | 13 | 56.5 |
Joint Damage | Hemophilia A | Hemophilia B | p | ||||
---|---|---|---|---|---|---|---|
Mild | Moderate | Severe | Mild | Moderate | Severe | ||
Right knee | 6.7% | 16.7% | 50.0% | 0.0% | 16.7% | 50.0% | 0.848 |
Left knee | 10.0% | 26.7% | 36.7% | 16.4% | 0.0% | 16.7% | 0.050 |
Right ankle | 16.7% | 30.0% | 30.0% | 83.3% | 16.7% | 0.0% | 0.007 |
Left ankle | 36.7% | 20.0% | 23.3% | 83.3% | 0.0% | 0.0% | 0.048 |
Right hip | 10.0% | 3.3% | 13.3% | 0.0% | 0.0% | 16.7% | 0.668 |
Left hip | 10.0% | 6.7% | 13.3% | 16.7% | 0.0% | 16.7% | 0.812 |
Right wrist | 20.0% | 13.3% | 13.3% | 0.0% | 16.7% | 0.0% | 0.223 |
Left wrist | 20.0% | 20.0% | 6.7% | 0.0% | 0.0% | 16.7% | 0.302 |
Right elbow | 16.7% | 30.0% | 23.3% | 16.7% | 16.7% | 0.0% | 0.214 |
Left elbow | 13.3% | 30.0% | 20.0% | 50.0% | 0.0% | 0.0% | 0.040 |
Right shoulder | 10.0% | 16.7% | 10.0% | 0.0% | 16.7% | 0.0% | 0.481 |
Left shoulder | 10.0% | 13.3% | 0.0% | 0.0% | 16.7% | 0.0% | 0.560 |
Joint Damage | Treatment | p | |||||
---|---|---|---|---|---|---|---|
Continuous Prophylaxis | Intermittent Prophylaxis | ||||||
Mild | Moderate | Severe | Mild | Moderate | Severe | ||
Right knee | 14.3% | 28.6% | 0.0% | 4.0% | 16.0% | 72.0% | 0.001 |
Left knee | 14.3% | 0.0% | 0.0% | 12.0% | 32.0% | 48.0% | 0.001 |
Right ankle | 42.9% | 28.6% | 0.0% | 28.0% | 32.0% | 36.0% | 0.050 |
Left ankle | 36.7% | 20.0% | 23.3% | 83.3% | 0.0% | 0.0% | 0.019 |
Right hip | 0.0% | 0.0% | 0.0% | 12.0% | 4.0% | 20.0% | 0.148 |
Left hip | 0.0% | 0.0% | 0.0% | 16.0% | 8.0% | 20.0% | 0.076 |
Right wrist | 0.0% | 0.0% | 0.0% | 24.0% | 20.0% | 16.0% | 0.014 |
Left wrist | 0.0% | 0.0% | 0.0% | 24.0% | 28.0% | 8.0% | 0.014 |
Right elbow | 14.3% | 14.3% | 0.0% | 20.0% | 36.0% | 28.0% | 0.025 |
Left elbow | 14.3% | 0.0% | 0.0% | 24.0% | 36.0% | 24.0% | 0.002 |
Right shoulder | 14.3% | 0.0% | 0.0% | 8.0% | 24.0% | 12.0% | 0.147 |
Left shoulder | 0.0% | 0.0% | 0.0% | 12.0% | 20.0% | 0.0% | 0.098 |
Comorbidities | Hemophilia A (n = 30) | Hemophilia B (n = 6) | Chi2 Test p | RR | IC95% | ||
---|---|---|---|---|---|---|---|
n | % | n | % | ||||
HCV infection | 20 | 66.7 | 3 | 50.0 | 0.369 | 1.77 A | 0.42–7.53 |
Arterial hypertension | 8 | 26.7 | 0 | 0.0 | 0.193 | 1.27 A | 1.05–1.54 |
Ischemic cardiac disease | 5 | 16.7 | 0 | 0.0 | 0.378 | 1.24 A | 1.04–1.47 |
Obesity | 5 | 16.7 | 2 | 33.3 | 0.329 | 1.21 B | 0.74–1.97 |
Gastrointestinal disease | 2 | 6.7 | 2 | 33.3 | 0.048 | 7.00 B | 0.76–6.46 |
Others | 4 | 13.3 | 0 | 0.0 | 0.465 | 1.23 A | 1.04–1.45 |
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Tarniceriu, C.C.; Hurjui, L.L.; Tanase, D.M.; Haisan, A.; Tepordei, R.T.; Statescu, G.; Vicoleanu, S.A.P.; Lupu, A.; Lupu, V.V.; Ursaru, M.; et al. Inherited Hemophilia—A Multidimensional Chronic Disease That Requires a Multidisciplinary Approach. Life 2025, 15, 530. https://doi.org/10.3390/life15040530
Tarniceriu CC, Hurjui LL, Tanase DM, Haisan A, Tepordei RT, Statescu G, Vicoleanu SAP, Lupu A, Lupu VV, Ursaru M, et al. Inherited Hemophilia—A Multidimensional Chronic Disease That Requires a Multidisciplinary Approach. Life. 2025; 15(4):530. https://doi.org/10.3390/life15040530
Chicago/Turabian StyleTarniceriu, Cristina Claudia, Loredana Liliana Hurjui, Daniela Maria Tanase, Anca Haisan, Razvan Tudor Tepordei, Gabriel Statescu, Simona Alice Partene Vicoleanu, Ancuta Lupu, Vasile Valeriu Lupu, Manuela Ursaru, and et al. 2025. "Inherited Hemophilia—A Multidimensional Chronic Disease That Requires a Multidisciplinary Approach" Life 15, no. 4: 530. https://doi.org/10.3390/life15040530
APA StyleTarniceriu, C. C., Hurjui, L. L., Tanase, D. M., Haisan, A., Tepordei, R. T., Statescu, G., Vicoleanu, S. A. P., Lupu, A., Lupu, V. V., Ursaru, M., & Nedelcu, A. H. (2025). Inherited Hemophilia—A Multidimensional Chronic Disease That Requires a Multidisciplinary Approach. Life, 15(4), 530. https://doi.org/10.3390/life15040530