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European Medicines Agency Pre-authorisation Evaluation of Medicines for Human Use Document Date: London, 9 February 2006 Doc.Ref.: EMEA/COMP/355971/2005 COMMITTEE FOR ORPHAN MEDICINAL PRODUCTS PUBLIC SUMMARY OF POSITIVE OPINION FOR ORPHAN DESIGNATION OF 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2-[(methylamino)carbonyl]hydrazine for the treatment acute myeloid leukaemia On 14 December 2005, orphan designation (EU/3/05/332) was granted by the European Commission to Vion (UK) Limited,, for 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2- [(methylamino)carbonyl]hydrazine for the treatment of acute myeloid leukaemia. What is acute myeloid leukaemia? Acute myeloid leukaemia is a disease in which cancer cells are found in the blood and the bone marrow. The bone marrow is the spongy tissue inside the large bones in the body. Normally, the bone marrow makes cells called blasts that mature into several different types of blood cells that have specific functions in the body. These include red cells, white cells and platelets. Red blood cells carry oxygen and other materials to all tissues of the body. White blood cells fight infection. Platelets make the blood clot. When leukaemia develops, the bone marrow produces large numbers of abnormal blood cells. There are several types of leukaemias. In myeloid leukaemia blasts that are developing into a type of white blood cells called granulocytes are affected. The blasts do not mature and become too many. These blast cells are then found in the blood and also accumulate in the bone marrow where they take the place of the other types of normal blood cells. When leukaemia develops quickly with many blasts it is called acute. Acute myeloid leukaemia is life-threatening. What are the methods of treatment available? Treatment for leukaemia is complex and depends on a number of factors including the type of leukaemia, the extent of the disease and whether the leukaemia has been treated before. It also depends on the age, the symptoms, and the general health of the patient. The primary treatment of acute myeloid leukaemia is chemotherapy (using drugs to kill cancer cells). Several products were authorised for the condition in the Community at the time of submission of the application for orphan drug designation. 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2-[(methylamino)carbonyl]hydrazine could be of potential significant benefit for the treatment of acute myeloid leukaemia because it might improve the longterm outcome of the patients. This assumption will have to be confirmed at the time of marketing authorisation. This will be necessary to maintain the orphan status. What is the estimated number of patients affected by the condition*? According to the information provided by the sponsor, acute myeloid leukaemia was considered to affect about 32,000 persons in the European Union. How is this medicinal product expected to act? 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2-[(methylamino)carbonyl]hydrazine is an alkylating agent. These agents interfere with the fundamental genetic material of the cells (DNA or RNA). They block tumour cell growth by forming cross-bridges in the DNA chains (DNA strands), which makes 7 Westferry Circus, Canary Wharf, London, E14 4HB, UK Tel. (44-20) 74 18 84 00 Fax (44-20) 75 23 70 40 E-mail: orphandrugs@emea.eu.int www.emea.eu.int Reproduction and/or distribution of this document is authorised for non-commercial purposes only provided the EMEA is acknowledged

the strands unable to uncoil and separate. This prevents DNA replication (making new copies) and thus the tumour cells can no longer divide. What is the stage of development of this medicinal product? The effects of 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2-[(methylamino)carbonyl]hydrazine were evaluated in experimental models. At the time of submission of the application for orphan designation, clinical trials in patients with acute myeloid leukaemia were ongoing. 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2-[(methylamino)carbonyl]hydrazine was not authorised anywhere worldwide for treatment of acute myeloid leukaemia, at the time of submission. Orphan designation of 1,2-bis(methylsulphonyl)-1-(2-chloroethyl)-2-[(methylamino)carbonyl]hydrazine was granted in the United States for treatment of acute myeloid leukaemia. According to Regulation (EC) No 141/2000 of 16 December 1999, the Committee for Orphan Medicinal Products (COMP) adopted on 24 October 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 Community) 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: Vion (UK) Limited i3 Research Sygnus Court 22-32 Market Street Maidenhead SL6 8AD Telephone: +44 16 28 40 84 08 Telefax: +44 16 28 40 84 01 E-mail: vionuk@vionpharm.co.uk *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. Page 2/5

Patients associations contact points: Leukaemia Care 2 Shrubbery Avenue Worcester WR1 1QH 0800 169 6680 (freephone for UK residents) Telephone: +44 19 05 33 00 03 / + 44 84 57 67 32 03 Telefax: +44 19 05 33 00 90 E-mail: enquiries@leukaemiacare.org.uk Leukaemia Research Fund 43 Great Ormond Street London WC1N 3JJ Telephone: +44 20 74 05 01 01 Telefax : +44 20 74 05 E-mail: info@lrf.org.uk Associazione Italiana contro le Leucemie-linfomi e mieloma ONLUS Via Ravenna 34 00161 Roma Italy Telephone: +39 06 44 03 763 Telefax: +39 06 44 04 226 E-mail: ail@ail.it Page 3/5

Translations of the active ingredient and indication in all EU languages and Norwegian and Icelandic Language Active Ingredient Indication English 1,2-bis(methylsulphonyl)-1-(2- chloroethyl)-2- [(methylamino)carbonyl]hydrazine Treatment of acute myeloid leukaemia Czech Danish Dutch Estonian Finnish French German Greek Hungarian Italian Latvian Lithuanian Polish Portuguese 1,2-bis(metylsulfonyl)-1-(2-chlóretyl)- 2-[(metylamino)karbonyl]hydrazin 1,2-bis(methylsulphonyl)-1-(2- chlorethyl)-2- [(methylamino)carbonyl]hydrazin 1,2-bis(methylsulfonyl)-1-(2- chloorethyl)-2- [(methylamino)carbonyl]hydrazine 1,2-bis(metüülsulfonüül)-1-(2- kloroetüül)-2- [(metüülamino)karbonüül]hüdrasiin 1,2-bis(metyylisulfonyyli)-1-(2- kloorietyyli)-2- [(metyyliamino)karbonyyli]hydratsiini 1,2-bis(méthylsulfonyl)-1-(2- chloroéthyle)-2- [(méthylamino)carbonyle]hydrazine 1,2-Bis(methylsulphonyl)-1-(2- chlorethyl)-2- [(methylamino)carbonyl]hydrazin 1,2-δις(µεθυλοσουλφονυλο)-1-(2- χλωροαιθυλο)-2- [(µεθυλαµινο)καρβινυλο]υδραζίνη 1,2-bisz(metilszulfonil)-1-(2-klór-etil)- 2-[(metilamino)karbonil]hidrazin [(metilammino)carbonil]idrazina 1,2-bis(metilsulfonīl)-1-(2-hloretīl)-2- [(metilamino)karbonil]hidrazīns 1,2-bis(metilsulfonil)-1-(2-chloretil)-2- [(metilamino)karbonil]hidrazinas 1,2-bis(metylosulfonylo)-1-(2- chloroetylo)-2- [(metyloamino)karbonylo]hydrazyna [(metilamino)carbonil]hidrazina Léčba akutní myeloidní leukémie Behandling af akut myeloid leukæmi Behandeling van acute myeloïde leukemie Akuutse müeloidse leukeemia ravi Akuutin myelooisen leukemian hoito Traitement de la leucémie aiguë myéloïde Behandlung der akuten myeloischen Leukämie Θεραπεία της οξείας µυελοειδούς λευχαιµίας Akut myeloid leukaemia kezelése Trattamento della leucemia mieloide acuta Akūtas mieloleikozes ārstēšana Ūmios mieloleukozės gydymas Leczenie ostrej białaczki szpikowej Tratamento da leucemia mielóide aguda Slovak 1,2-bis(metylsulfonyl)-1-(2-chloroetyl)- Liečba akútnej myeloickej leukémie Page 4/5

Slovenian Spanish Swedish Norwegian Icelandic 2-[(metylamíno)karbonyl]hydrazín 1,2-bis(metilsulfonil)-1-(2-hloroetil)-2- [(metilamino)karbonil]hidrazin [(metilamino)carbonil]hidracina 1,2-bis(metylsulfonyl)-1-(2-kloretyl)-2- [(metylamin)karbonyl]hydrazin 1,2-bis(metylsulfonyl)-1-(2-kloretyl)-2- [(metylamino)karbonyl]hydrazin 1,2-bis(metýlsúlfónýl)-1-(2-klóróetýl)- 2-[(metýlamínó)karbónýl]hýdrazín Zdravljenje akutne mieloične levkemije Tratamiento de la leucemia mieloide aguda Behandling av akut myeloisk leukemi Behandling av akutt myelogen leukemi Meðferð við bráðu kyrningahvítblæði Page 5/5