Swedish Orphan Biovitrum and Pharming sign Rhucin® distribution agreement

Stockholm, Sweden and Leiden, The Netherlands - Swedish Orphan Biovitrum (STO: BVT) and Pharming Group NV (NYSE Euronext: PHARM) today announced an exclusive 10 year distribution agreement, under which Swedish Orphan Biovitrum will distribute Rhucin® in 24 EU countries, Norway, Iceland and Switzerland.

Under the agreement, Swedish Orphan Biovitrum will pay to Pharming an undisclosed upfront payment and a regulatory approval milestone. In addition, Swedish Orphan Biovitrum will pay a supply price that will include tiered performance based royalties to Pharming. Swedish Orphan Biovitrum will also have the right to participate in the future development and distribution of Rhucin® in the agreed countries for additional indications.

Rhucin® is a recombinant human C1 inhibitor for the acute treatment of Hereditary Angioedema (HAE) attacks. Pharming submitted an MAA for Rhucin® to the European Medicines Agency (EMA) in September 2009.

“Rhucin® is a novel medicine that offers great value to HAE patients. This orphan drug fits perfectly with our business goals and in our product portfolio. We are very much looking forward to a longstanding partnership with Pharming and the respective growth of both companies. We are delighted to provide what we believe will be a highly beneficial medicine to patients who need it,” said Martin Nicklasson, CEO of Swedish Orphan Biovitrum Group.

“We are confident that we will successfully bring Rhucin® through the remainder of the regulatory review process and look forward to making Rhucin® available for HAE patients in Europe shortly thereafter. With their impressive track record in selling and distributing medicines in the rare disease area in Europe, Swedish Orphan Biovitrum makes an ideal partner to maximize the value of our product in a mutually beneficial collaboration,” said Dr. Sijmen de Vries, Chief Executive Officer of Pharming.



About Rhucin® (conestat alfa)
Rhucin® or recombinant human C1 inhibitor (rhC1INH) is a recombinant protein for acute treatment of Hereditary Angioedema (HAE) attacks. Rhucin® has identical amino acid sequence as endogenous human C1INH. The safety and efficacy of Rhucin® has been demonstrated in two placebo controlled and four open-label studies. Both randomized placebo-controlled clinical trials showed statistically significant and clinically relevant improvement in time to relief of symptoms and time to minimal symptoms compared to placebo. Rhucin® holds an orphan drug designation both from the US FDA and EMA.



About Hereditary Angioedema
Hereditary Angioedema (HAE) is a debilitating and potentially life-threatening genetic condition, resulting in spontaneous and recurring attacks of angioedema. Angioedema attacks are characterized by swelling of soft tissue in a reaction visually similar to severe allergic reactions. Attacks may affect various locations such as the intestines, the face, extremities or mouth and throat. Without treatment, attacks progress during the first 24 hours and then subside over another two to five days. Attacks are painful and disfiguring and cause significant morbidity. In addition to the life-threatening nature of the disease, quality of life for individuals with the disease may be seriously impaired.

HAE is caused by the lack or deficiency of endogenous C1INH activity, which results in an overreaction of the immune system. Patients typically present in late childhood, with the mean onset at 11 years. The prevalence of HAE is approximately 1 in 30.000 individuals. The frequency of attacks varies significantly in the affected population. Current treatment options include prophylactic treatment with tranexamic acid and androgens, which may reduce the frequency of attacks. The average frequency even under such anabolic steroid prophylaxis is still estimated to be 6-8 treatment requiring attacks per year. For the treatment of acute attacks, the current standard of care in Europe is plasma derived C1 inhibitor from human donors. Swedish Orphan Biovitrum estimates the current value of the European HAE market to approximately €110 million and sees the potential of the HAE market to increase with the introduction of Rhucin® as one of several new treatment alternatives.



About Swedish Orphan Biovitrum
On January 14, 2010, Biovitrum AB (publ) completed the acquisition of Swedish Orphan International Holding AB and created Swedish Orphan Biovitrum - a leading company focused on treatment of rare diseases.

Swedish Orphan Biovitrum is a Swedish based specialty pharmaceutical company with an international market presence. The company is focused on providing and developing orphan and niche specialist pharmaceuticals to patients with high medical needs. The portfolio consists of about 60 marketed products and an emerging late stage clinical development pipe-line within rare diseases. Swedish Orphan Biovitrum has pro-forma revenues 2009e of about 2 BSEK and approximately 500 employees. The head office is located in Sweden and the share (STO: BVT) is listed on NASDAQ OMX Stockholm. For more information please visit www.biovitrum.com.



About Pharming Group NV
Pharming Group NV is developing innovative products for the treatment of genetic disorders, ageing diseases, specialty products for surgical indications, and nutritional products. Pharming’s lead product Rhucin® for acute attacks of Hereditary Angioedema has passed clinical development stage and the Market Authorization Application is under review with the European Medicines Agency. Prodarsan® is in early stage clinical development for Cockayne Syndrome and lactoferrin for use in food products. The advanced technologies of the Company include innovative platforms for the production of protein therapeutics, technology and processes for the purification and formulation of these products, as well as technology in the field of DNA repair (via DNage). Additional information is available on the Pharming website, www.pharming.com

This press release contains forward looking statements that involve known and unknown risks, uncertainties and other factors, which may cause the actual results, performance or achievements of the Company to be materially different from the results, performance or achievements expressed or implied by these forward looking statements.