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European Medicines Agency Pre-authorisation Evaluation of Medicines for Human Use Document Date: London, 18 August 2008 Doc.Ref.: EMEA/OMP/193391/2005 Rev.1 OMMITTEE FOR ORPHAN MEDIINAL PRODUTS PUBLI SUMMARY OF POSITIVE OPINION FOR ORPHAN DESIGNATION OF hepatitis immunoglobulin for the prevention of recurrent hepatitis virus induced liver disease in liver transplant recipients On 8 July 2005 orphan designation (EU/3/05/295) was granted by the European ommission to Nabi Biopharmaceuticals Europe Ltd, Ireland, for hepatitis immunoglobulin for the prevention of recurrent hepatitis virus induced liver disease in liver transplant recipients. The sponsorship was transferred to Biotest Pharma GmbH, Germany, in February 2008. What is recurrent hepatitis virus induced liver disease in liver transplant recipients? Hepatitis is a general term that means inflammation of the liver. Hepatitis is an infectious disease that affects the liver, caused by the hepatitis virus. This viral infection does not always manifest itself clinically, and thus some people infected with the virus will not look or feel ill, others might have mild flu-like symptoms, or can become very ill. Most patients fully recover spontaneously while others remain infected, initially without any sign or symptom of the disease. They are called hepatitis virus carriers. Hepatitis carriers are at risk of serious liver disease, such as cirrhosis (liver scarring) and liver cancer. In these cases of advanced liver disease, liver transplantation (getting a liver from another person) could become a necessity. However, since the virus is found also in body fluids (such as blood) of hepatitis virus carriers, the transplanted liver might become infected on its turn and the liver disease can start in the transplanted liver. Recurrent hepatitis virus induced liver disease in liver transplant recipients is chronically debilitating and life-threatening. What are the methods of prevention available? No satisfactory methods exist that were authorised at the time of application What is the estimated number of patients at risk of developing the condition *? Based on the information provided by the sponsor and previous knowledge of the ommittee, the number of patients at risk of recurrent hepatitis virus induced liver disease in liver transplant recipients was considered to be less than 1 in 10,000 persons in the European Union, which, at the time of designation, corresponded to about 46,000 persons. * Disclaimer: For the purpose of the designation, the number of patients affected by the condition is estimated and assessed based on data from the European Union (EU 25), Norway, Iceland and Lichtenstein. This represents a population of 459,700,000 (Eurostat 2004). This estimate is based on available information and calculations presented by the sponsor at the time of the application. 7 Westferry ircus, anary Wharf, London, E14 4HB, UK Tel. (44-20) 74 18 84 00 Fax (44-20) 75 23 70 40 E-mail: orphandrugs@emea.europa.eu http://www.emea.europa.eu European Medicines Agency, 2008. Reproduction is authorised provided the source is acknowledged

How is this medicinal product expected to act? Antibodies are proteins, which specifically recognise and attach themselves to certain structures, such as proteins found on the surface of viruses or bacteria. Hepatitis immunoglobulin is an antibody that recognises, attaches and neutralises the hepatitis virus. In experimental models it has been shown that hepatitis immunoglobulin would prevent recurrent hepatitis virus induced liver disease. Therefore, if administered after liver transplantation, it would protect the new liver from the harmful activity of the hepatitis virus. What is the stage of development of this medicinal product? The effects of hepatitis immunoglobulin were evaluated in experimental models. At the time of submission of the application for orphan designation, clinical trials in patients who received liver transplantation due to chronic hepatitis liver disease were initiated. Hepatitis immunoglobulin was not authorised anywhere worldwide for recurrent hepatitis virus induced liver disease in liver transplant recipients, at the time of submission. Orphan designation of hepatitis virus immune globulin (human) was granted in the United States for the condition of prophylaxis of hepatitis infection in liver transplant recipients. According to Regulation (E) No 141/2000 of 16 December 1999, the ommittee for Orphan Medicinal Products (OMP) adopted on 12 May 2005 a positive opinion recommending the grant of the above-mentioned designation. Opinions on orphan medicinal products designations are based on the following cumulative criteria: (i) the seriousness of the condition, (ii) the existence or not of alternative methods of diagnosis, prevention or treatment and (iii) either the rarity of the condition (considered to affect not more than five in ten thousand persons in the ommunity) or the insufficient return of development investments. Designated orphan medicinal products are still investigational products which were considered for designation on the basis of potential activity. An orphan designation is not a marketing authorisation. As a consequence, demonstration of the quality, safety and efficacy will be necessary before this product can be granted a marketing authorisation. For more information: Sponsor s contact details: Biotest Pharma GmbH Landsteinerstr. 5 63303 Dreieich Germany Telephone: + 49 6103 801 588 Telefax: + 49 6103 801 180 E-mail: mail@biotest.de Page 2/5

Patients associations contact points: Euroliver Foundation Markgravestraat, 10 P.O. B-2000 Anvers Belgium Telephone: +32 3 226 79 97 Telefax: +32 3 226 79 70 E-mail: euroliver@glo.be British Liver Trust Portman House 44 High Street Ringwood BH24 1AG United Kingdom Telephone: +44 14 25 46 30 80 Telefax: +44 14 25 47 07 06 Page 3/5

Translations of the active ingredient and indication in all EU languages and Norwegian and Icelandic Language Active Ingredient Indication English Hepatitis Immunoglobulin Prevention of recurrent hepatitis virus induced liver disease in liver transplant recipients zech Hepatitis Immunoglobulin Prevence recidivy jaterního onemocnění indukovaného virem hepatitidy u nemocných po transplantaci jater Danish Hepatitis Immunoglobulin Forebyggelse mod tilbagevendende hepatitis virus induceret leversygdom i levertransplantatmodtagere Dutch Hepatitis immunoglobine Preventie van wederkerende hepatitis virus geïnduceerde leveraandoening in levertransplant recipiënten Estonian -hepatiidi immuunoglobuliin Retsidiivse -hepatiidist tingitud maksahaiguste ärahoidmine maksatransplantaadi retsipientidel. Finnish Hepatiitti- immunoglobuliini Toistuvan hepatiitti -viruksen aiheuttaman maksasairauden estäminen maksansiirtojen vastaanottajissa French Immunoglobuline anti-hépatite Prévention des manifestations hépatiques récurrentes causées par le virus de l'hépatite chez les transplantés hépatiques German Hepatitis Immunoglobulin Schutz vor Wiederauftreten der Hepatitis - Infektion bei Lebertransplantatempfängern Greek Ανοσοσφαιρίνη Ηπατίτιδας Πρόληψη από υποτροπιάζουσα ηπατική νόσο προερχόμενη από τον ιό της Ηπατίτιδας σε λήπτες μοσχεύματος ήπατος Hungarian Hepatitis- immunoglobulin Májtranszplantációt követő visszatérő Hepatitis virus fertőzés megelőzése Italian Immunoglobulina dell Epatite Prevenzione delle malattie epatiche ricorrenti indotte dal virus dell epatite in pazienti sottoposti a trapianto di fegato Latvian Hepatīta imūnglobulīns Atkārtotas hepatīta izraisītas aknu slimības profilaksei aknu transplantāta recipientiem Lithuanian Hepatito imunoglobulinas Kepenų ligos, nulemtos pakartotinio hepatito viruso, prevencija kepenų transplanto recipientams Polish Portuguese Slovak Immunoglobulina przeciw zapaleniu wątroby typu Imunoglobulina para a Hepatite Imunoglobulín hepatitídy typu Zapobieganie nawracającemu zapaleniu wątroby wywołanemu przez wirus typu u biorców przeszczepu wątroby Prevenção da doença hepática recorrente induzida pelo vírus da hepatite em recipientes de transplante hepático Prevencia rekurentnej hepatitídy u príjemcov transplantátov pečene s vírusmi indukovanými ochoreniami pečene Slovenian Hepatitis Immunoglobulin Preprečevanje ponovne jetrne okvare, povzročene s HV, pri prejemniku jetrnega transpantata Page 4/5

Spanish Inmunoglobulina de Hepatitis Prevención de la recurrencia de la enfermedad hepática inducida por el virus de la hepatitis en receptores transplante de hígado Swedish hepatit immunglobulin Profylax av återkommande hepatit virusinducerad lever sjukdom hos levertransplantat mottagare Norwegian Immunoglobulin mot hepatitt Forebygging av tilbakevendende hepatitt virus-indusert leversykdom hos levertransplanterte pasienter Icelandic Lifrarbólgu ónæmisglóbúlín Fyrirbyggjandi gegn endurteknum lifrarbólgu tengdum lifrarsjúkdómi hjá lifrarígræðsluþegum Page 5/5