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

Authors: Mahn, R., Schilling, K., Klamroth, R., Kentouche, K., Aumann, V., Fischer, L., Holzhauer, S., Sirb, H., Scholz, U., Trautmann, K., Halm-Heinrich, I., Krammer-Steiner, B., Koscielny, J., Kreibich, U., Pietrzak-Buttner, A., Tregel, M., Knofler, R., and Pfrepper, C.

Publication: Hamostaseologie 2020; 40,1:119-127.

Affiliations: Univ Hosp Leipzig, Med Dept 1, Div Haemostaseol, Liebigstr 20, D-04103 Leipzig, Germany ; Univ Hosp Jena, Div Haematol & Oncol, Jena, Germany ; Vivantes Hosp Friedrichshain, Berlin, Germany ; Univ Hosp Magdeburg, Dept Paediat, Magdeburg, Germany ; Charite, Dept Paediat Haematol & Oncol, Berlin, Germany ; DRK Hosp Lichtenstein, Dept Paediat, Lichtenstein, Germany ; Ctr Coagulat Disorders, Leipzig, Germany ; Univ Hosp Dresden, Dept Haematol & Oncol, Dresden, Germany ; Rostock South City Med Ctr, Dept Haematol & Oncol, Rostock, Germany ;Heinrich Braun Hosp, Haemophilia Ambulance, Zwickau, Germany ; SRH Hosp Suhl, Paediat Clin, Suhl, Germany ; Ruppin Gen Hosp, Brandenburg Med Sch, Inst Lab Med, Neuruppin, Germany.

Abstract: INTRODUCTION: In 2005 the Kompetenznetz Hamorrhagische 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.