Main Article Content

Mario Schiavoni



Background: The health-related quality of life (HRQoL) of people with hemophilia (PWH) is an important issue, especially considering people suffering from chronic diseases beyond hemophilia. The principal aim of this study was to investigate the presence and relevance of psychological symptoms, both internalizing and externalizing, lifestyle, and HRQoL in a group of Italian PWH with chronic bloodborne co-infections and comorbidities. Furthermore, the research describes the association between psychological aspects and the impact of disease-related characteristics (type of hemophilia, presence of co-infections, and comorbidities) on them.

Methods: Seventy patients (mean age 46.77±11.3), 64 with severe hemophilia A (Factor VIII: C < 1 IU/dL) and 6 with severe hemophilia B (Factor IX <1 IU/dL), were consecutively recruited from seven Hemophilia Centers in Italy of Italian Association of Hemophilia Centers (AICE). In order to assess psychological symptoms, HRQoL, and lifestyle, three psychological questionnaires were administered (the SCL-90-R, SF-36, and PSQ, respectively).

Results: A general decline in the quality of life and an increase in the tendency to adopt a lifestyle characterized by hyperactivity emerged. Inverse correlations were found between HRQoL and psychological distress. Although the SCL-90-R did not reveal symptoms above the clinical cut-off, co-infections significantly increased anxiety, depression, somatizations, paranoia, and social withdrawal. Lastly, HRQoL is impaired by co-infections as well as comorbidities.

Conclusion: Our preliminary results must be confirmed to deepen the findings between mental health and hemophilia.


Download data is not yet available.

Abstract 720
PDF Downloads 872
HTML Downloads 317


1. Davari M, Gharibnaseri Z, Ravanbod R, Sadeghi A. Health status and quality of life in pa-tients with severe hemophilia A: A cross-sectional survey. Hematol Rep,2019;14;11(2):7894. doi: 10.4081/hr.2019.7894.
2. Torres-Ortuño A, Cuesta-Barriuso R, Nieto-Munuera J, Castiello-Munuera Á, López-Pina JA. Validation of the Spanish Version of the VERITAS-PRN Scale to Assess Adherence to on Demand Regimens in Patients with Hemophilia. Patient Prefer Adherence,2021;11;15:967-974. doi: 10.2147/PPA.S310662.
3. Bago M, Butkovic A, Preloznik Zupan I, Faganel Kotnik B, Prga I, Bacic Vrca V, Zupancic Salek S. Association between reported medication adherence and health-related quality of life in adult patients with haemophilia. Int J Clin Pharm,2021;43(6):1500-1507. doi: 10.1007/s11096-021-01270-x.
4. Mohan R, Radhakrishnan N, Varadarajan M, Anand S. Assessing the current knowledge, attitude and behaviour of adolescents and young adults living with haemophilia. Haemophilia,2021;27(2):e180-e186. doi: 10.1111/hae.14229.
5. Buckner TW, Sidonio R Jr, Witkop M, Guelcher C, Cutter S, Iyer NN, Cooper DL. Corre-lations between patient-reported outcomes and self-reported characteristics in adults with hemophilia B and caregivers of children with hemophilia B: analysis of the B-HERO-S study. Patient Relat Outcome Meas,2019;10:299-314. doi: 10.2147/PROM.S219166.
6. Holstein K, von Mackensen S, Bokemeyer C, Langer F. The impact of social factors on outcomes in patients with bleeding disorders. Haemophilia,2016;22(1):46-53. doi: 10.1111/hae.12760.
7. Cutter S, Molter D, Dunn S, Hunter S, Peltier S, Haugstad K, Frick N, Holot N, Cooper DL. Impact of mild to severe hemophilia on education and work by US men, women, and caregivers of children with hemophilia B: The Bridging Hemophilia B Experiences, Results and Opportunities into Solutions (B-HERO-S) study. Eur J Haematol, 2017;98 Suppl 86:18-24. doi: 10.1111/ejh.12851.
8. F R M Y Cassis, A Buzzi, A Forsyth, M Gregory, D Nugent, C Garrido, T Pilgaard, D L Cooper, A Iorio. Haemophilia Experiences, Results and Opportunities (HERO) Study: in-fluence of haemophilia on interpersonal relationships as reported by adults with haemophilia and parents of children with haemophilia. Haemophilia, 2014;20(4):e287-95.
9. Isfordink CJ, van Erpecum KJ, van der Valk M, Mauser-Bunschoten EP, Makris M. Viral hepatitis in haemophilia: historical perspective and current management. Br J Haema-tol,2021;195(2):174-185. doi: 10.1111/bjh.17438.
10. Lowe GD. Haemophilia, blood products and HIV infection. Scott Med J,1987;32(4):109-11. doi: 10.1177/003693308703200404.
11. Barry V, Lynch ME, Tran DQ, Antun A, Cohen HG, DeBalsi A, Hicks D, Mattis S, Ribeiro MJ, Stein SF, Truss CL, Tyson K, Kempton CL. Distress in patients with bleeding disor-ders: a single institutional cross-sectional study. Haemophilia,2015;21(6):e456-64. doi: 10.1111/hae.12748.
12. Drugs for HIV infection. The Medical Letter on Drugs and Therapeutics. Haemophilia, 2000;6, Suppl.1:73-89.
13. Dew MA, Ragni MV, Nimorwicz P. Infection with human immunodeficiency virus and vulnerability to psychiatric distress. A study of men with hemophilia. Arch Gen Psychia-try,1990;47(8):737-44. doi: 10.1001/archpsyc.1990.01810200045006.
14. Catalan J, Klimes I, Bond A, Day A, Garrod A, Rizza C. The psychosocial impact of HIV infection in men with haemophilia: controlled investigation and factors associated with psychiatric morbidity. J Psychosom Res,1992;36(5):409-16. doi: 10.1016/0022-3999(92)90001-i.
15. Marsettin EP, Ciavarella N, Lobaccaro C, Ghirardini A, Bellocco R, Schinaia N. Psycho-logical status of men with haemophilia and HIV infection: two-year follow-up. Haemophi-lia,1995;1(4):255-61. doi: 10.1111/j.1365-2516.1995.tb00085.x.
16. Gil F, Arranz P, Lianes P, Breitbart W. Physical symptoms and psychological distress among patients with HIV infection. AIDS Patient Care,1995;9(1):28-31. doi: 10.1089/apc.1995.9.28.
17. Tzitzikos G, Kotrotsiou E, Bonotis K, Gourgoulianis K. Assessing hostility in patients with chronic obstructive pulmonary disease (COPD). Psychol Health Med, 2019; 24 (5): 605-619. doi:10.1080/13548506.2018.1554253.
18. Oz HS, Oz F. A psychoeducation program for stress management and psychosocial prob-lems in multiple sclerosis. Niger J Clin Pract. 2020;23(11):1598-1606. doi:10.4103/njcp.njcp_462_19
19. Grassi L, Belvederi Murri M, Riba M, et al. Hostility in cancer patients as an underexplored facet of distress. Psychooncology. 2021;30(4):493-503. doi:10.1002/pon.5594
20. Apgáua LT, Jaeger A. Memory for emotional information and alexithymia A systematic review. Dement Neuropsychol, 2019;13(1):22-30. doi: 10.1590/1980-57642018dn13-010003.
21. Derogatis LR. SCL-90-R: Administration, Scoring and Procedures Manual. Minneapolis, MN. National Computer Systems, 1994.
22. Sarno I, Preti E, Prunas A, Madeddu F. SCL-90-R Symptom Checklist-90-R Adattamento italiano. Firenze: Giunti, Organizzazioni Speciali, 2011.
23. Derogatis LR. Misuse of the symptom checklist 90. Arch Gen Psychiat-ry,1983;40(10):1152-3. doi: 10.1001/archpsyc.1983.01790090114025.
24. Apolone G, Mosconi P. The Italian SF-36 Health Survey: translation, validation and norming. J Clin Epidemiol,1998;51(11):1025-36. doi: 10.1016/s0895-4356(98)00094-8.
25. Pruneti C. The « P Stress Questionnaire »: A new tool for the evaluation of stress-related behaviours, European Journal of Clinical Psychology and Psychiatry,2011;VI, 3: 1-37.
26. Pilowsky I, Spence ND. Manual for the illness behavior questionnaire (IBQ), (3rd Edn). Adelaide: University of Adelaide, 1994.
27. Gringeri A, Leissinger C, Cortesi PA, Jo H, Fusco F, Riva S, Antmen B, Berntorp E, Biaso-li C, Carpenter S, Kavakli K, Morfini M, Négrier C, Rocino A, Schramm W, Windyga J, Zülfikar B, Mantovani LG. Health-related quality of life in patients with haemophilia and inhibitors on prophylaxis with anti-inhibitor complex concentrate: results from the Pro-FEIBA study. Haemophilia, 2013;19(5):736-43. doi: 10.1111/hae.12178.
28. Cuesta-Barriuso R, Torres-Ortuño A, Nieto-Munuera J, López-Pina JA. Quality of Life, Perception of Disease and Coping Strategies in Patients with Hemophilia in Spain and El Salvador: A Comparative Study. Patient Prefer Adherence,2021;15:1817-1825. doi: 10.2147/PPA.S326434.
29. Bago M, Butkovic A, Faganel Kotnik B, Prga I, Bačić Vrca V, Zupančić Šalek S, Preloznik Zupan I. Health-Related Quality of Life in Patients with Haemophilia and Its Association with Depressive Symptoms: A Study in Croatia and Slovenia. Psychiatr Dan-ub,2021;33(3):334-341. doi: 10.24869/psyd.2021.334.
30. Pinto PR, Paredes AC, Moreira P, Fernandes S, Lopes M, Carvalho M, Almeida A. Emo-tional distress in haemophilia: Factors associated with the presence of anxiety and depres-sion symptoms among adults. Haemophilia,2018;24(5):e344-e353. doi: 10.1111/hae.13548.
31. Banchev A, Batorova A, Faganel Kotnik B, Kiss C, Puras G, Zapotocka E, Zupancic-Salek S; Patient Advocacy Group. A Cross-National Survey of People Living with Hemophilia: Impact on Daily Living and Patient Education in Central Europe. Patient Prefer Adherence, 2021;15:871-883.
32. Kempton CL, Makris M, Holme PA. Management of comorbidities in haemophilia. Hae-mophilia. 2021 Feb;27 Suppl 3:37-45. doi: 10.1111/hae.14013.
33. Zawilska K, Podolak-Dawidziak M. Therapeutic problems in elderly patients with hemo-philia. Pol Arch Med Wewn,2012;122(11):567-76.
34. Franzoni F, Scarfò G, Guidotti S, Fusi J, Asomov M, Pruneti C. Oxidative Stress and Cog-nitive Decline: The Neuroprotective Role of Natural Antioxidants. Front Neuro-sci,2021;15:729757. doi: 10.3389/fnins.2021.729757.
35. Torres-Ortuño A, Cuesta-Barriuso R, Nieto-Munuera J, Galindo-Piñana P, López-Pina JA. Coping strategies in young and adult haemophilia patients: A tool for the adaptation to the disease. Haemophilia. 2019 May;25(3):392-397
36. Sumari-de Boer IM, Sprangers MA, Prins JM, Nieuwkerk PT. HIV stigma and depressive symptoms are related to adherence and virological response to antiretroviral treatment among immigrant and indigenous HIV infected patients. AIDS Behav,2012;16(6):1681-9. doi: 10.1007/s10461-011-0112-y.
37. Carrico AW, Bangsberg DR, Weiser SD, Chartier M, Dilworth SE, Riley ED. Psychiatric correlates of HAART utilization and viral load among HIV-positive impoverished persons. AIDS,2011;25(8):1113-8. doi: 10.1097/QAD.0b013e3283463f09.
38. Limperg PF, Maurice-Stam H, Heesterbeek MR, Peters M, Coppens M, Kruip MJHA, Eik-enboom J, Grootenhuis MA, Haverman L. Illness cognitions associated with health-related quality of life in young adult men with haemophilia. Haemophilia,2020;26(5):793-799. doi: 10.1111/hae.14120.
39. Trindade GC, Viggiano LGL, Brant ER, Lopes CAO, Faria ML, Ribeiro PHNS, Silva AFDC, Souza DMR, Lopes AF, Soares JMA, Pinheiro MB. Evaluation of quality of life in hemophilia patients using the WHOQOL-bref and Haemo-A-Qol questionnaires. Hematol Transfus Cell Ther,2019;41(4):335-341. doi: 10.1016/j.htct.2019.03.010.
40. Yuvaraj A, Mahendra VS, Chakrapani V, Yunihastuti E, Santella AJ, Ranauta A, Doughty J. HIV and stigma in the healthcare setting. Oral Dis.,2020;26 Suppl 1:103-111. doi: 10.1111/odi.13585.
41. Nanni MG, Caruso R, Mitchell AJ, Meggiolaro E, Grassi L. Depression in HIV infected patients: a review. Curr Psychiatry Rep,2015;17(1):530. doi: 10.1007/s11920-014-0530-4.
42. Ciccarelli N, Fabbiani M, Grima P, Falasca K, Tana M, Baldonero E, Colafigli M, Silveri MC, Vecchiet J, Cauda R, Di Giambenedetto S. Comparison of cognitive performance in HIV or HCV mono-infected and HIV-HCV co-infected patients. Infec-tion,2013;41(6):1103-9. doi: 10.1007/s15010-013-0503-2.
43. Dalgard O, Egeland A, Skaug K, Vilimas K, Steen T. Health-related quality of life in active injecting drug users with and without chronic hepatitis C virus infection. Hepatolo-gy,2004;39(1):74-80. doi: 10.1002/hep.20014.
44. Rosendaal FR, Smit C, Varekamp I, Bröcker-Vriends A, Suurmeijer TP, Briët E. AIDS and haemophilia. A study among Dutch haemophiliacs on the psychological impact of the AIDS threat, the prevalence of HIV antibodies and the adoption of measures to prevent HIV transmission. Haemostasis,1988;18(2):73-82. doi: 10.1159/000215786.
45. Miličić D, Brajković L, Maček JL, Andrić A, Ardalić Ž, Buratović T, Marčinko D. Type a Personality, Stress, Anxiety and Health Locus of Control in Patients with Acute Myocardial Infarction. Psychiatr Danub,2016;28(4):409-414.
46. Bonaguidi F, Michelassi C, Trivella MG, Carpeggiani C, Pruneti CA, Cesana G, L'Abbate A. Cattell's 16 PF and PSY inventory: relationship between personality traits and behavior-al responses in patients with acute myocardial infarction. Psychol Rep,1996;78(2):691-702. doi: 10.2466/pr0.1996.78.2.691.
47. Cosentino C, Sgromo D, Merisio C, Berretta R, Pruneti C. Psychophysiological Adjustment to Ovarian Cancer: Preliminary Study on Italian Women Condition. Appl Psychophysiol Biofeedback,2018;43(2):161-168. doi: 10.1007/s10484-018-9395-3.
48. De Vincenzo F, Cosentino C, Quinto RM, Di Leo S, Contardi A, Guidotti S, Iani L, Pruneti C. Psychological adjustment and heart rate variability in ovarian cancer survivors. Mediterr J Clin Psychol,2022; 10. https://doi.org/10.13129/2282-1619/mjcp-3318.
49. Morris G, Reiche EMV, Murru A, Carvalho AF, Maes M, Berk M, Puri BK. Multiple Im-mune-Inflammatory and Oxidative and Nitrosative Stress Pathways Explain the Frequent Presence of Depression in Multiple Sclerosis. Mol Neurobiol,2018;55(8):6282-6306. doi: 10.1007/s12035-017-0843-5.
50. Kallaur AP, Lopes J, Oliveira SR, Simão AN, Reiche EM, de Almeida ER, Morimoto HK, de Pereira WL, Alfieri DF, Borelli SD, Kaimen-Maciel DR, Maes M. Immune-Inflammatory and Oxidative and Nitrosative Stress Biomarkers of Depression Symptoms in Subjects with Multiple Sclerosis: Increased Peripheral Inflammation but Less Acute Neu-roinflammation. Mol Neurobiol,2016;53(8):5191-202. doi: 10.1007/s12035-015-9443-4.