2014-03-26

Latest marketing authorisations and orphan drug designations



Marketing authorisations and medicinal products designations

Detailed information on European orphan medicinal products designation applications is available on theEMA website. A full list of designated and authorised orphan medicinal products in Europe available at:ec.europa.eu.

Learn more about the Orphan designation process in Europe

Recent orphan drug designations

February 2014

Treatment of Farber disease
Recombinant human acid ceramidase

Prevention of congenital cytomegalovirus infection following primary cytomegalovirus infection
Mixture of recombinant human IgG1 monoclonal antibodies against human cytomegalovirus envelope glycoproteins

Treatment of epidermolysis bullosa
Diacerein

Diagnosis of gastro-entero-pancreatic neuroendocrine tumours
Gallium [Ga-68]-N-[(4,7,10-tricarboxymethyl-1,4,7,10-tetraazacyclododec-1-yl)acetyl]-D-phenylalanyl-L-cysteinyl-L-tyrosyl-D-tryptophanyl-L-lysyl-L-threoninyl-Lcysteinyl-L-threonine-cyclic(27)disulfide

Prevention of delayed graft function after solid organ transplantation
Eculizumab

Treatment of cystic fibrosis
11-(4-Dimethylamino-3-hydroxy-6-methyl-tetrahydro-pyran-2-yloxy)-2-ethyl-3,4,10-trihydroxy-3,5,6,8,10,12,14-heptamethyl-1-oxa-6-azacyclopentadecane-13,15-dione

Treatment of cystic fibrosis
Cysteamine

Treatment of Duchenne muscular dystrophy
Asp-Arg-Val-Tyr-Ile-His-Pro

Treatment of Rett síndrome
3-Chloro-4-fluorophenyl-[4-fluoro-4-{[(5-methylpyrimidin-2-ylmethyl)amino]methyl}piperidin-1-yl]methanone

Treatment of polycythaemia vera
Ruxolitinib

Treatment of adrenoleukodystrophy
Pioglitazone

Diagnosis of gastro-entero-pancreatic neuroendocrine tumours
68Ga-2,2'-(7-(4-((S)-1-((4S,7S,10S,13R,16S,19R)-4-((R)-1-amino-3-(4-hydroxyphenyl)-1-oxopropan-2-ylcarbamoyl)-10-(4-aminobutyl)-16-(4-((S)-2,6-dioxohexahydropyrimidine-4-carboxamido)benzyl)-7-((R)-1-hydroxyethyl)-6,9,12,15,18-pentaoxo-13-(4-ureidobenzyl)-1,2-dithia-5,8,11,14,17-pentaazacycloicosan-19-ylamino)-3-(4-chlorophenyl)-1-oxopropan-2-ylamino)-1-carboxy-4-oxobutyl)-1,4,7-triazonane-1,4-diyl)diacetic acid

Treatment of glioma
Autologous dendritic cells pulsed with tumour antigen-derived synthetic peptides (MAGE-1, HER-2, AIM-2, TRP-2, gp-100, and interleukin-13 receptor alpha)

Treatment of optic neuritis
N-({Carbamoylmethyl-[3-(2-oxo-pyrrolidin-1-yl)-propyl]-carbamoyl}-methyl)-2-[2-(2-fluoro-phenyl)-ethylamino]-N-isobutyl-acetamide

Treatment of familial chylomicronemia syndrome
Phosphorothioate oligonucleotide targeted to apolipoprotein CIII

January 2014

Treatment of malignant mesothelioma
Amatuximab

Treatment of chronic lymphocytic leukaemia / small lymphocytic lymphoma
Inecalcitol

Treatment of aneurysmal subarachnoid haemorrhage
Sodium nitrite

Treatment of hepatitis delta virus infection
Lonafarnib

Treatment of dystrophic myotonia
(6aS)-1,10-dimethoxy-6-methyl-5,6,6a,7-tetrahydro-4Hdibenzo[de,g]quinoline-2,9-diol

Treatment of adenovirus infection in allogeneic haematopoietic stem-cell transplant recipients
Adenovirus-specific T-cells derived from allogeneic donor leukocytes, expanded ex vivo

Treatment of primary sclerosing cholangitis
Obeticholic acid

Treatment of malignant mesothelioma
Autologous dendritic cells pulsed with allogeneic tumour cell lysate

Treatment of acute myeloid leukaemia
(2R,3R,4S,5R)-2-(6-amino-9H-purin-9-yl)-5-((((1r,3S)-3-(2-(5-(tert-butyl)-1H benzo[d]imidazol-2-yl)ethyl)cyclobutyl)(isopropyl) amino) methyl)tetrahydrofuran-3,4-diol

Treatment of acute lymphoblastic leukaemia
(2R,3R,4S,5R)-2-(6-amino-9H-purin-9-yl)-5-((((1r,3S)-3-(2-(5-(tert-butyl)-1Hbenzo[d]imidazol-2-yl)ethyl)cyclobutyl)(isopropyl)amino)methyl)tetrahydrofuran-3,4-diol

Treatment of epidermolysis bullosa
Allantoin

Prevention of graft-versus-host disease
Allogeneic bone-marrow derived ex-vivo expanded multipotent adult progenitor cells

Treatment of chronic lymphocytic leukaemia / small lymphocytic lymphoma
N-(3-(5-fluoro-2-(4-(2-methoxyethoxy)phenylamino)pyrimidin-4-ylamino) phenyl)acrylamide benzenesulfonic acid salt
 

December 2013

Treatment of cystic fibrosis
Nitric oxide

Treatment of hypoparathyroidism
Recombinant human parathyroid hormone

Treatment of glioma
Allogeneic and autologous haptenised and irradiated cells and cell lysates derived from glioma

Treatment of follicular lymphoma
Ibrutinib

Prevention of necrotizing enterocolitis
Lactobacillus acidophilus and Bifidobacterium bifidum

Treatment of Alagille síndrome
 (4R,5R)-1-[[4-[[4-[3,3-dibutyl-7-(dimethylamino)-2,3,4,5-tetrahydro-4-hydroxy-1,1-dioxido-1-benzothiepin-5-yl]phenoxy]methyl]phenyl]methyl]-4-aza-1-azoniabicyclo[2.2.2]octane chloride

Treatment of primary biliary cirrosis
 (4R,5R)-1-[[4-[[4-[3,3-dibutyl-7-(dimethylamino)-2,3,4,5-tetrahydro-4-hydroxy-1,1-dioxido-1-benzothiepin-5-yl]phenoxy]methyl]phenyl]methyl]-4-aza-1-azoniabicyclo[2.2.2]octane chloride

Treatment of progressive familial intrahepatic cholestasis
 (4R,5R)-1-[[4-[[4-[3,3-dibutyl-7-(dimethylamino)-2,3,4,5-tetrahydro-4-hydroxy-1,1-dioxido-1-benzothiepin-5-yl]phenoxy]methyl]phenyl]methyl]-4-aza-1-azoniabicyclo[2.2.2]octane chloride

Treatment of primary sclerosing colangitis
 (4R,5R)-1-[[4-[[4-[3,3-dibutyl-7-(dimethylamino)-2,3,4,5-tetrahydro-4-hydroxy-1,1-dioxido-1-benzothiepin-5-yl]phenoxy]methyl]phenyl]methyl]-4-aza-1-azoniabicyclo[2.2.2]octane chloride

Prevention of arteriovenous access dysfunction in haemodialysis patients
Recombinant human type I pancreatic elastase

Treatment of Dravet syndrome
Fenfluramine hydrochloride

Treatment of dengue
Poly[2-[(4-{[1-carboxy-2-(hexadecylcarbamoyl)ethyl]sulfanyl}-2,3-bis({2-[((2S)-2-(2-{[(2R)-2-carbamoyl-(2-{[(2S)-1-ethoxy-3-(3-hydroxy-4oxo-1,4-dihydropyridin-1-yl)-1-oxopropan-2-yl]carbamoyl}ethyl]sulfanyl}-3-{[(2S)-1-ethoxy-3-(3-hydroxy-4-oxo-1,4-dihydropyridin-1-yl)-1-oxopropan-2-yl]carbamoyl}propanamido)-3-(3-hydroxy-4-oxo-1,4-dihydropyridin-1-yl)propanoyl Ethyl ester) )-methoxy]acetyl}oxy)butyl)sulfanyl]-3-(hexadecylcarbamoyl)propanoic acid]-poly(ethylene glycol)-ester]

Treatment of haemophilia A
Humanised monoclonal modified IgG4 antibody with bispecific structure targeting factors IX, IXa, X and Xa

November 2013

Treatment of glioma
Autologous ex-vivo-expanded leucocytes treated with 5-aza-2’-deoxycytidine

Treatment of mucopolysaccharidosis type II (Hunter's syndrome)
Recombinant human insulin receptor monoclonal antibody-fusediduronate 2-sulfatase

Treatment of follicular thyroid cancer
Sorafenib tosylate

Treatment of papillary thyroid cancer
Sorafenib tosylate

Prevention of graft-versus-host disease
Defibrotide

Treatment of hepatocellular carcinoma
Tivantinib

Treatment of diffuse large B-cell lymphoma
Ibrutinib

Prevention of arteriovenous access dysfunction in patients undergoing surgical creation of an arteriovenous access for haemodialysis
Sirolimus

Treatment of eosinophilic oesophagitis
Human monoclonal antibody against human interleukin 13

Treatment of spinal cord injury
Synthetic 12 amino acids peptide designed after subcommissural organ spondin

Treatment of ovarian cancer
Trebananib

Treatment of Stargardt’s disease
Soraprazan

October 2013

Treatment of congenital factor VII deficiency
Recombinant fusion protein linking coagulation factor VIIa with Albumin

Treatment of myotonic disorders
Mexiletine hydrochloride

Treatment of plasma cell myeloma
Recombinant human monoclonal IgM antibody targeting glucose-regulated protein 78

Treatment of sarcoidosis
L-Pyr-L-Glu-L-Gln-L-Leu-L-Glu-L-Arg-L-Ala-L-Leu-L-Asn-LSer-L-Ser

Treatment of complex regional pain syndrome
Zoledronic acid

Treatment of Allan-Herndon-Dudley syndrome
3,5-diiodothyropropionic acid

Treatment of Duchenne muscular dystrophy
Naproxcinod

Treatment of cystic fibrosis
Antisense oligonucleotide targeting the F508delta mutation of CFTR

Treatment of Wiskott-Aldrich-syndrome
Autologous CD34+ cells transduced with a lentiviral vector containing the human Wiskott-Aldrich syndrome gene

July & August 2013

Treatment of pulmonary alveolar proteinosis
Granulocyte macrophage colony stimulating factor

Autologous bone marrow-derived mesenchymal stromal cells secreting neurotrophic factors
Autologous bone marrow-derived mesenchymal stromal cells secreting neurotrophic factors

Prevention of ischaemia/reperfusion injury associated with solid organ transplantation
Human hemin

Treatment of B-lymphoblastic leukaemia/lymphoma
Moxetumomab pasudotox

Treatment of malignant thymomas
Belinostat

Treatment of pancreatic cancer
(1-methyl-2-nitro-1H-imidazole-5-yl) methyl N,N’-bis(2-bromoethyl) diamidophosphate

Treatment of plasma cell myeloma
Daratumumab

Treatment of ovarian cancer
Fosbretabulin tromethamine

Treatment of amyotrophic lateral sclerosis
Allogeneic motor neuron progenitor cells derived from human embryonic stem cells

Prevention of hepatitis B re-infection following liver transplantation
Recombinant human monoclonal antibody against hepatitis B Virus

Treatment of follicular lymphoma
(S)-3-(1-(9H-purin-6-ylamino)ethyl)-8-chloro-2-phenylisoquinolin-1(2H)-one

Treatment of ataxia telangiectasia
Dexamethasone sodium phosphate encapsulated in human autologous erythrocytes

Treatment of carbamoylphosphate synthase-1 deficiency
Heterologous human adult liver-derived progenitor cells

Treatment of citrullinaemia type 1
Heterologous human adult liver-derived progenitor cells

Treatment of argininosuccinic aciduria
Heterologous human adult liver-derived progenitor cells

Treatment of hyperargininaemia
Heterologous human adult liver-derived progenitor cells

Treatment of N-acetylglutamate synthetase (NAGS) deficiency
Heterologous human adult liver-derived progenitor cells

Treatment of citrullinaemia type 2
Heterologous human adult liver-derived progenitor cells

Treatment of ornithine translocase deficiency (hyperornithinaemia-hyperammonaemia homocitrullinuria (HHH) syndrome)
Heterologous human adult liver-derived progenitor cells

Treatment of limbal stem cell deficiency
Ex-vivo expanded autologous human corneal epithelium containing stem cells

Treatment of osteosarcoma
Lipid-complexed cisplatin

Treatment of acromegaly
Octreotide acetate (oral use)

Treatment of nodal marginal zone lymphoma
Idelalisib

Treatment of chronic lymphocytic leukaemia/small lymphocytic lymphoma
Idelalisib

Treatment of acute myeloid leukaemia
Trans-N1-((1R,2S)-2-phenylcyclopropyl)cyclohexane-1,4-diamine bis-hydrochloride

Treatment of autosomal dominant polycystic kidney disease
Tolvaptan

Treatment of nontraumatic osteonecrosis
Human allogeneic bone marrow derived osteoblastic-like cells

Treatment of pancreatic cancer
Chimeric monoclonal antibody against claudin-18 splice variant 2

Treatment of Pseudomonas aeruginosa lung infection in cystic fibrosis
Pegylated recombinant anti-Pseudomonas aeruginosa PcrV Fab′antibody

Treatment of growth hormone deficiency
Recombinant human growth hormone modified by fusion with two hydrophilic polypeptide chains

Treatment of Behçets' disease
Apremilast

Treatment of eosinophilic oesophagitis
Budesonide

Treatment of mastocytosis
Cladribine

Treatment of congenital sucrase-isomaltase deficiency
Sacrosidase

Treatment of sickle cell disease
(1R,3R,4R,5S)-3-O-[2-O-benzoyl-3-O-(sodium(2S)-3-cyclohexyl-propanoate-2-yl)-β-D-galactopyranosyl]-4-O-(α-L-fucopyranosyl)-5-orothylamido-cyclohexane-1-carboxylic acid ethyl-2-amidyl-ethyloxy-2-acetyl-(8-amino-1,3,6-naphthalene-tris sodium sulfonate) amide

Treatment of neuromyelitis optica
Eculizumab

Treatment of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
Idelalisib

Treatment of splenic marginal zone lymphoma
Idelalisib
 

June 2013

Treatment of cystic fibrosis
4,6,4’–trimethylangelicin

Treatment of 5q spinal muscular atrophy
5-[1-(2,6-dichlorobenzyl)piperidin-4-ylmethoxy]quinazoline-2,4-diamine dihydrochloride

Treatment of amyotrophic lateral sclerosis
Sodium chlorite

Treatment of retinitis pigmentosa
Expanded human allogeneic neural retinal progenitor cells extracted from neural retina

Treatment of pachyonychia congenital
Synthetic double-stranded siRNA oligonucleotide directed against the keratin 6a N171K mutation

Treatment of retinitis pigmentosa
Adenovirus associated viral vector serotype 5 containing the human pde6β gene

Treatment of acute liver failure
Immortalised human C3A hepatoblastoma cells

Treatment of mucopolysaccharidosis type IIIB (Sanfilippo B syndrome)
Recombinant human alpha-N-acetylglucosaminidase

Treatment of soft tissue sarcoma
Genetically modified serotype 5/3 adenovirus coding for granulocyte-macrophage colony-stimulating factor

Treatment of retinitis pigmentosa
Unoprostone isopropyl

Treatment of non-dystrophic myotonia
Mexiletine hydrochloride

Treatment of B-cell acute lymphoblastic leukaemia
Inotuzumab ozogamicin

Treatment of adrenocortical carcinoma
N-[2,6-bis(1-methylethyl)phenyl]-N’-[[1-[4-(dimethylamino)phenyl]cyclopentyl]methyl]urea, hydrochloride salt

Treatment of graft-versus-host disease
Allogeneic bone marrow derived mesenchymal cells expanded ex vivo in synthetic media

Treatment of transglutaminase-1-deficient autosomal recessive congenital ichthyosis
Recombinant human transglutaminase 1 encapsulated into liposomes

Treatment of cystic fibrosis
Recombinant human CXCL8 mutant

Treatment of malignant mesothelioma
N-methyl-4-({4-[({3-methyl(methylsulfonyl)aminopyrazin-2-yl}methyl)amino]-5-(trifluoromethyl)pyrimidin-2-yl}amino)benzamide hydrochloride

Treatment of cytomegalovirus disease in patients with impaired cell mediated immunity
Maribavir

Treatment of adenosine deaminase-deficient-severe combined immunodeficiency
Autologous CD34+ cells transduced with a lentiviral vector containing the human ADA gene

Treatment of retinitis pigmentosa
Recombinant human nerve growth factor
 

April 2013

Treatment of follicular thyroid cancer
Lenvatinib

Treatment of glioma
4-[2-(6-methylpyridin-2-yl)-5,6-dihydro-4H-pyrrolo[1,2-b]pyrazol-3-yl]-quinoline-6-carboxamide monohydrate

Treatment of papillary thyroid cancer
Lenvatinib

Treatment of Duchenne muscular dystrophy
R,S-O-(3-piperidino-2-hydroxy-1-propyl)-nicotinic acid amidoxime dihydrochloride

Treatment of idiopathic pulmonary fibrosis
Nintedanib

Treatment of Niemann-Pick disease, type C
2-hydroxypropyl-β-cyclodextrin

Treatment of chronic lymphocytic leukaemia/small lymphocytic lymphoma
(S)-3-(1-(9H-purin-6-ylamino)ethyl)-8-chloro-2-phenylisoquinolin-1(2H)-one

March 2013

Treatment of neuronal ceroid lipofuscinosis type 2
Recombinant human tripeptidyl-peptidase 1

Treatment of Stargardt’s disease
Ramiprilat

Treatment of Churg-Strauss Syndrome
Mepolizumab

Treatment of mantle cell lymphoma
1-[(3R)-3-[4-amino-3-(4-phenoxyphenyl)-1H- pyrazolo [3,4-d]pyrimidin-1-yl]-1-piperidinyl]-2-propen-1-one

Treatment of congenital alpha-1 antitrypsin deficiency
Cyclo[L-alanyl-L-seryl-L-isoleucyl-L-prolyl-L-prolyl-Lglutaminyl-L-lysyl-L-tyrosyl-D-prolyl-L-prolyl-(2S)-2-aminodecanoyl-L-alpha-glutamyl-L-threonyl] acetate salt

Prevention of graft rejection following solid organ transplantation
Murine IgM monoclonal antibody binding to alpha beta T-cell receptor

Treatment of sickle cell disease
Poloxamer 188

Treatment of chronic non-infectious uveitis
Gevokizumab

Treatment of Niemann-Pick disease, type C
Recombinant human heat shock protein 70

Treatment of hepatocellular carcinoma
4-[2-(6-methylpyridin-2-yl)-5,6-dihydro-4H-pyrrolo[1,2-b]pyrazol-3-yl]-quinoline-6-carboxamide monohydrate

Treatment of systemic sclerosis
2-[4-Methoxy-3-(2-m-tolyl-ethoxy)-benzoylamino]-indan-2-carboxylic acid

February 2013

Treatment of achromatopsia caused by mutations in the CNGB3 gene
Recombinant adeno-associated viral vector containing the human CNGB3 gene

Treatment of amyloid light-chain amyloidosis
Humanised IgG1 kappa antibody against serum amyloid A and AL amyloid

Treatment of moderate and severe traumatic brain injury
Progesterone

Treatment of high altitude pulmonary oedema
Cyclo-Cys-Gly-Gln-Arg-Glu-Thr-Pro-Glu-Gly-Ala-Glu-Ala-Lys-Pro-Trp-Tyr-Cys

Treatment of chronic thromboembolic pulmonary hypertension
Treprostinil sodium

Treatment of systemic sclerosis
Terguride

Treatment of Duchenne muscular dystrophy
Humanised monoclonal antibody against myostatin

Treatment of acute myeloid leukaemia
L-asparaginase encapsulated in erythrocytes

January 2013

Treatment of follicular lymphoma
Lenalidomide

Treatment of familial adenomatous polyposis
Eflornithine in combination with sulindac

Treatment of growth hormone deficiency
Recombinant modified human growth hormone

Treatment of 5q spinal muscular atrophy
Allogeneic motor neuron progenitor cells derived from human embryonic stem cells

Treatment of Wilson’s disease
Choline tetrathiomolybdate

Treatment of pancreatic cancer
Recombinant human monoclonal antibody of the IgG1 kappa class against prostate stem cell antigen

Treatment of beta-thalassaemia intermedia and major
Autologous CD34+ haematopoietic stem cells transduced with lentiviral vector encoding the human betaA-T87Q-globin gene

Treatment of ovarian cancer
Chimeric monoclonal antibody against claudin 6

Treatment of glioma
1,2:5,6-Dianhydrogalactitol

Treatment of achondroplasia
Modified recombinant human C-type natriuretic peptide

Treatment of mucopolysaccharidosis type IIIB (Sanfilippo B syndrome)
Adeno-associated viral vector serotype 9 containing the human N-acetylglucosaminidase alpha gene

Treatment of systemic sclerosis
Terguride

Treatment of retinitis pigmentosa
Encapsulated human retinal pigment epithelial cell line transfected with plasmid vector expressing human ciliary neurotrophic factor

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