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Review
. 2020 Feb;40(1):119-127.
doi: 10.1055/s-0039-3399493. Epub 2019 Nov 11.

Development of Haemophilia Treatment in the Eastern Part of Germany over the Last Decade in the Kompetenznetz Hämorrhagische Diathese Ost (KHDO)

Rebecca Mahn  1 , Kristina Schilling  2 , Robert Klamroth  3 , Karim Kentouche  4 , Volker Aumann  5 , Lars Fischer  6 , Susanne Holzhauer  7 , Harry Sirb  8 , Ute Scholz  9 , Karolin Trautmann  10 , Ines Halm-Heinrich  11 , Beate Krammer-Steiner  12 , Jürgen Koscielny  13 , Ute Kreibich  14 , Antje Pietrzak-Büttner  15 , Matthias Tregel  16 , Ralf Knöfler  17 , Christian Pfrepper  1 ; and the Kompetenznetzwerk Hämorrhagische Diathese Ost
Affiliations
Review

Development of Haemophilia Treatment in the Eastern Part of Germany over the Last Decade in the Kompetenznetz Hämorrhagische Diathese Ost (KHDO)

Rebecca Mahn et al. Hamostaseologie. 2020 Feb.

Abstract
in English, German

Introduction: In 2005 the Kompetenznetz Hämorrhagische Diathese Ost published epidemiologic data about patients with haemophilia A (HA) and haemophilia B (HB) in the eastern part of Germany. This study provides data about the development of treatment in these patients over the past 10 years.

Methods: Data from 12 haemophilia centres in eastern Germany were retrospectively collected for the year 2015 from patients' records.

Results: We evaluated 413 patients (115 children, 298 adults) with HA or HB. A total of 286 patients (69.2%) had severe haemophilia (patients with severe haemophilia, PWSH). Compared with 2005, the proportion PWSH on prophylaxis increased from 90% to 98.8% in children and from 64% to 80.2% in adults. The use of plasma-derived factor concentrates decreased from >70% to 55.3% in children and to 55.1% in adults. Mean annual factor consumption in PWSH without inhibitor was higher in 2015 compared with 2005 (children with HA: 151,489 vs. 98,894; adults with HA: 217,151 vs. 151,394; children with HB: 105,200 vs. 64,256; adults with HB: 159,185 vs. 85,295). Median annualized bleeding (annualized bleeding rate, ABR) and joint bleeding rates (annualized joint bleeding rate, AJBR) in 2015 were 2 and 0 in children and 3 and 0 in adults, respectively. In 2015 only one child (1.2%) but 101 (53.2%) adults with severe haemophilia were anti-hepatitis C virus (anti-HCV) positive. The rate of anti-HCV positive patients with active hepatitis C dropped from 63.8% to 12.9%.

Conclusions: Within the last decade more patients with severe haemophilia were switched to a prophylactic regimen going along with a moderate increase in factor consumption achieving a low ABR and AJBR.

Einleitung: Im Jahr 2005 hat das Kompetenznetz Hämorrhagische Diathese Ost epidemiologische Daten über Patienten im Osten Deutschlands mit Hämophilie A (HA) und B (HB) publiziert. Diese Studie liefert Daten über die Entwicklung der Hämophiliebehandlung bei diesen Patienten in den letzten 10 Jahren.

Methoden: Daten von 12 Hämophiliezentren im Osten Deutschlands aus dem Jahr 2015 wurden retrospektiv aus den Patientenakten ausgewertet.

Ergebnisse: Wir untersuchten 413 Patienten (115 Kinder, 298 Erwachsene) mit HA oder HB. 286 Patienten (69,2%) hatten eine schwere Hämophilie (PMSH). Im Vergleich zu 2005 ist der Anteil der PMSH mit Prophylaxe bei den Kindern von 90% auf 98.8% und bei den Erwachsenen von 64% auf 80,2% angestiegen. Der Verbrauch von plasmatischen Faktorenkonzentraten ist von >70% auf 55,3% bei Kindern und 55,1% bei Erwachsenen gesunken. Der mittlere Faktorenverbrauch bei PMSH ohne Hemmkörper war 2015 höher als 2005 (Kinder mit HA 151.489 versus 98.894, Erwachsene mit HA 217.151 versus 151.394, Kinder mit HB 105.200 versus 64.256, Erwachsenen mit HB 159.185 versus 85.295). Die mediane jährliche Blutungsrate und Gelenkblutungsrate in 2015 war 2 bzw. 0 bei Kindern und 3 bzw. 0 bei Erwachsenen. Im Jahr 2015 war lediglich ein Kind (1,2%) aber 101 (53,2%) Erwachsene mit schwerer Hämophilie anti-HCV positiv. Der Anteil der anti-HCV-positiven Erwachsenen mit aktiver Hepatitis C fiel von 63,8% auf 12,9%.

Zusammenfassung: In den letzten 10 Jahren wurden mehr Patienten auf eine prophylaktische Behandlung umgestellt. Dies geht mit einem moderaten Anstieg des Verbrauchs an Faktorenkonzentraten einher, resultiert aber in einer niedrigen Blutungsrate.

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Conflict of interest statement

Adj. Prof. Dr. J. Koscielny declares the following conflicts of interest: speaker honoraria from Aspen, Bayer Health Care Pharmaceuticals, Daiichi Sankyo, Boehringer Ingelheim, CSL Behring, Sanofi-Aventis, Shire, Roche, Chugai, Pfizer, BMS, Mitsubishi Pharma, Ferring GmbH, Sanofi-Aventis and Novo Nordisk. Adj. Prof. Dr. J. Koscielny is also a medical advisor for CSL Behring International, Bayer HealthCare Pharmaceuticals (national and international), Chugai, Roche, Shire (national) and Novo Nordisk (national) during the last 3 years. Dr. Halm-Heinrich reports personal fees and non-financial support from Novo Nordisk Deutschland, non-financial support from CSL Behring Deutschland, non-financial support from Shire Deutschland Gmbh, non-financial support from Bayer Vital GmbH, non-financial support from Biotest AG, non-financial support from LFB GmbH, outside the submitted work. Dr. Klamroth reports grants and personal fees from Bayer, grants and personal fees from CSL Behring, grants and personal fees from Roche, grants and personal fees from Pfizer, grants and personal fees from Biomarin, grants and personal fees from Takeda/Shire, grants and personal fees from Novo Nordisk, grants and personal fees from SOBI, outside the submitted work. Dr. Schilling reports non-financial support from Bayer Vital GmbH, personal fees from LEO Pharma GmbH, personal fees from Novo Nordisk Pharma GmbH, personal fees from Swedish Orphan Biovitrum GmbH, non-financial support from Roche Pharma AG, outside the submitted work. Prof. Knöfler reports grants and lecture honorarium from Bayer Vital GmbH, Novo Nordisk GmbH, Shire Deutschland GmbH, CSL Behring GmbH, Intersero GmbH, Biotest AG and Swedish Orphan Biovitrum GmbH during the 36 months prior to publication. Dr. Tregel reports other from Grifols, grants from Octapharma, other from Shire, outside the submitted work. Dr. Pfrepper reports and Dr. C. Pfrepper declares the following conflicts of interest: speaker honoraria from BMS, Pfizer, Roche, Shire and CSL Behring. Dr. C. Pfrepper is also a medical advisor for CSL Behring, Bayer HealthCare, Roche, Chugai, Shire, Novo Nordisk and Pfizer during the last 3 years.