Social/economic costs and health-related quality of life in patients with histiocytosis in Europe

  • Georgi IskrovEmail author
  • Itziar Astigarraga
  • Rumen Stefanov
  • Julio López-Bastida
  • Renata Linertová
  • Juan Oliva-Moreno
  • Pedro Serrano-Aguilar
  • Manuel Posada-de-la-Paz
  • Arrigo Schieppati
  • Domenica Taruscio
  • Márta Péntek
  • Johann Matthias Graf von der Schulenburg
  • Panos Kanavos
  • Karine Chevreul
  • Ulf Persson
  • Giovanni Fattore
  • BURQOL-RD Research Network
Original Paper



The aim of this study was to determine the economic burden from a societal perspective and the health-related quality of life (HRQOL) of patients with histiocytosis in Europe.


We conducted a cross-sectional study of patients with histiocytosis from France, Germany, Italy, Spain, Bulgaria, the UK, and Sweden. Data on demographic characteristics, health resource utilisation, informal care, loss of labour productivity and HRQOL were collected from the questionnaires completed by patients or their caregivers. HRQOL was measured with the EuroQol 5-domain (EQ-5D) questionnaire.


A total of 134 patients (35 France, 32 Germany, 30 Italy, 24 Spain, 7 Bulgaria, 4 UK and 2 Sweden) completed the questionnaire. The average annual costs ranged from € 6832 to € 33,283 between countries, the year of reference being 2012. Estimated direct healthcare costs ranged from € 1698 to € 18,213; direct nonhealthcare costs ranged from € 2936 to € 17,622 and labour productivity losses ranged from € 1 to € 8855. The mean EQ-5D score for adult histiocytosis patients was estimated at between 0.32 and 0.85, and the mean EQ-5D visual analogue scale score was estimated at between 50.00 and 66.50.


The main strengths of this study lie in our bottom-up approach to costing and in the evaluation of histiocytosis patients from a broad perspective (societal costs). This type of analysis is very scarce in international literature for rare diseases in comparison with other illnesses. We conclude that histiocytosis patients incur considerable societal costs and experience substantial deterioration in HRQOL.


Histiocytosis Cost-of-illness Social cost Health-related quality of life Europe 



The authors wish to thank: National Alliance of People with Rare Diseases (NAPRD), Bulgaria; Alliance Maladies Rares, France; ACHSE, Germany; Hungarian Federation of People with Rare and Congenital Diseases (RIROSZ), Hungary; Federazione Italiana Malattie Rare (UNIAMO), Italy; Consulta Nazionale delle Malattie Rare, Italy; AIRI LCH Onlus - Associazione Italiana Ricerca Istiocitos, Italyi; Rare Diseases Sweden; Federación Española de Efermedades Raras (FEDER), Spain; Rare Disease UK; Euro-Histio-Net and Rare Diseases Europe (EURORDIS); Euro-Histio-Net; Association Histiocytose France; Histiozytosehilfe e.V., Germany; AIRI LCH Onlus– Associazione Italiana Ricerca Istiocitosi, Italy; Föräldraföreningen för barn med Histiocytos, Sweden; Asociación Española contra la Histiocitosis, Spain.

Compliance with ethical standards


Supported by the Social Economic Burden and Health-Related Quality of Life in Patients with Rare Diseases in Europe Project, which has received funding from the European Union in the framework of the Health Programme [grant A101205]. The Executive Agency of the European Union is not responsible for any use that may be made of the information contained here.

Conflict of interest

The authors declare that they have no conflicts of interest.

Supplementary material

10198_2016_790_MOESM1_ESM.docx (16 kb)
Supplementary material 1 (DOCX 16 kb)
10198_2016_790_MOESM2_ESM.doc (41 kb)
Supplementary material 2 (DOC 41 kb)


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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Georgi Iskrov
    • 1
    • 17
    Email author
  • Itziar Astigarraga
    • 2
  • Rumen Stefanov
    • 1
    • 17
  • Julio López-Bastida
    • 3
    • 4
  • Renata Linertová
    • 4
    • 5
  • Juan Oliva-Moreno
    • 4
    • 6
  • Pedro Serrano-Aguilar
    • 4
    • 7
  • Manuel Posada-de-la-Paz
    • 8
  • Arrigo Schieppati
    • 9
  • Domenica Taruscio
    • 10
  • Márta Péntek
    • 11
  • Johann Matthias Graf von der Schulenburg
    • 12
  • Panos Kanavos
    • 13
  • Karine Chevreul
    • 14
    • 18
    • 19
  • Ulf Persson
    • 15
  • Giovanni Fattore
    • 16
  • BURQOL-RD Research Network
  1. 1.Institute for Rare DiseasesPlovdivBulgaria
  2. 2.Department of Pediatrics, BioCruces Health Research InstituteHospital Universitario CrucesBizkaiaSpain
  3. 3.University of Castilla-La ManchaTalavera de la Reina, ToledoSpain
  4. 4.Red de Investigación en Servicios Sanitarios en Enfermedades Crónicas (REDISSEC)MadridSpain
  5. 5.Fundación Canaria de Investigación Sanitaria (FUNCANIS)Las Palmas de Gran CanariaSpain
  6. 6.University of Castilla-La ManchaToledoSpain
  7. 7.Evaluation and Planning Service at Canary Islands Health ServiceSanta Cruz de TenerifeSpain
  8. 8.Institute of Rare Diseases ResearchISCIII, SpainRDR & CIBERERMadridSpain
  9. 9.Centro di Ricerche Cliniche per Malattie Rare Aldo e Cele DaccòIstituto di Ricerche Farmacologiche Mario NegriBergamoItaly
  10. 10.National Centre for Rare DiseasesIstituto Superiore di Sanità (ISS)RomeItaly
  11. 11.Department of Health EconomicsCorvinus University of BudapestBudapestHungary
  12. 12.Centre for Health Economics Research Hannover (CHERH)Leibniz Universität HannoverHannoverGermany
  13. 13.Department of Social Policy and LSE HealthLondon School of Economics and Political ScienceLondonUK
  14. 14.URC Eco Ile de FranceAP-HP, Hôtel DieuParisFrance
  15. 15.The Swedish Institute for Health EconomicsLundSweden
  16. 16.Centre for Research on Health and Social Care Management (CERGAS)Bocconi UniversityMilanItaly
  17. 17.Department of Social Medicine and Public Health, Faculty of Public HealthMedical University of PlovdivPlovdivBulgaria
  18. 18.Université Paris Diderot, Sorbonne Paris Cité, ECEVE, UMRS 1123ParisFrance
  19. 19.INSERM, ECEVE, U1123ParisFrance

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