Pharming Group N.V.

Regulatory Filings • Jan 24, 2012

NEW DATA PUBLISHED ON RUCONEST'S EFFECT ON BLOOD CLOTTING PARAMETERS DURING HAE ATTACKS

Leiden, The Netherlands, January 24, 2012. Biotech company Pharming Group NV ("Pharming" or "the Company") (NYSE Euronext: PHARM) announced today that recombinant human C1 inhibitor (rhC1INH; RUCONEST®) was not observed to have a prothrombotic effect when used to treat acute Hereditary Angioedema (HAE) attacks in a study published by Relan et al in the peer-reviewed journal Biodrugs.

These results can be found online and will be published in print on 1 February 2012 in Biodrugs issue 26:1.

Thrombotic events have been reported at the recommended dose of plasma-derived C1 inhibitor products following treatment of HAE. To investigate the effect of RUCONEST on blood clotting parameters, blood samples of 25 HAE patients experiencing an angioedema attack included in a randomized clinical trial were analyzed for levels of coagulation (clotting) and fibrinolytic (clot dissolution) parameters before and after infusion of saline placebo, RUCONEST 50 U/kg, or RUCONEST 100 U/kg.

The findings in this study suggest that HAE patients feature changes in coagulation and fibrinolytic parameters during an HAE attack. Administration of RUCONEST dose-dependently restored the balance in the intrinsic coagulation pathway and resulted in a reduction of thrombin generation. No harmful effect was observed either on other coagulation or fibrinolysis activation parameters.

Pharming's Chief Medical Officer, Dr. Rienk Pijpstra, commented: "These findings suggest that, contrary to being thrombogenic, in this study RUCONEST did not have any negative effect on the clotting parameters."

About RUCONEST (RHUCIN in non-European territories) and Hereditary Angioedema

RUCONEST® (INN conestat alfa) is a recombinant version of the human protein C1 inhibitor (C1INH). RUCONEST is produced through Pharming's proprietary technology in milk of transgenic rabbits and in Europe is approved under the name RUCONEST for treatment of acute angioedema attacks in patients with HAE. RHUCIN® is an investigational drug in the U.S. and has been granted orphan drug designation for the treatment of acute attacks of HAE, a genetic disorder in which the patient is deficient in or lacks a functional plasma protein C1 inhibitor, resulting in unpredictable and debilitating episodes of intense swelling of the extremities, face, trunk, genitals, abdomen and upper airway. The frequency and severity of HAE attacks vary and are most serious when they involve laryngeal edema, which can close the upper airway and cause death by asphyxiation. According to the U.S. Hereditary Angioedema Association, epidemiological estimates for HAE range from one in 10,000 to one in 50,000 individuals.

About Pharming Group NV

Pharming Group NV is developing innovative products for the treatment of unmet medical needs. RUCONEST® (RHUCIN® in non-European territories) is a recombinant human C1 inhibitor approved for the treatment of angioedema attacks in patients with HAE in all 27 EU countries plus Norway, Iceland and Liechtenstein, and is distributed in the EU by Swedish Orphan Biovitrum (OMX: SOBI). RHUCIN® is partnered with Santarus, Inc (NASDAQ: SNTS) in North America where the drug is undergoing Phase III clinical development. The product is also being evaluated for follow-on indications in the areas of transplantation and reperfusion injury. The advanced technologies of the Company include innovative and validated platforms for the production of protein therapeutics, technology and processes for the purification and formulation of these products. Recently a new project, using the validated transgenic rabbit platform, aimed at the development of recombinant Factor VIII for the treatment of Haemophilia A was initiated. 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.

Contact

Karl Keegan, CFO: T: +31 6 3168 0465 Rienk Pijpstra, CMO: T: +31 71 52 47 400

FTI Consulting Julia Phillips/ John Dineen, T: +44 (0)207 269 7193

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