Good afternoon, ladies and gentlemen. I'm sitting here with Chief Financial Officer, Jeroen Wakkerman to take you through the half year results 2021. But before I do that, next slide, please, I would like to draw your attention to the slide that you will see in front of you now, showing -- the slide on forward-looking statements, because we will be making some forward-looking statements that are based upon our current plans or believes, and that may change from time-to-time, as it's impossible to predict any new risks and uncertainties that may emerge from time-to-time. So having said that, let's go and have a look at the next slide please. And whilst that slide is coming up, I'll start introducing the company. So we are a revenue generating and profitable dual listed biopharmaceutical company and we're really investing at this point in time in building up our company towards the long-term. That means we are investing in the in development of recombinant human proteins from our own platform. And of course, our lead product is in this case, recombinant human C1 esterase inhibitor, RUCONEST, which is approved for the treatment of acute angioedema attacks in patients with hereditary angioedema, and where we have additional indications and clinical developments, but we're also investing in extensions of in-licensed development pipeline, we in-licensed late-stage compound leniolisib for the treatment of APDS from Novartis about a good year and a half ago, that is late-stage. And we recently in-license an early stage, ex-vivo hematopoietic stem cell therapy from Orchard Therapeutics for the potential treatment of hereditary angioedema. And we are -- and we can do this from our own means. That means we are profitable from revenues from our own commercial infrastructure and selling RUCONEST in the US and in the European Union. And…

Yeah. Thank you very much Sijmen. As you will remember, we saw in Q1 2021, the U.S. healthcare economy significantly affected by the second wave of COVID-19. And that also had an impact on our results in Q1. We saw our turnaround though, in Q2 where doctors' offices reopened and yeah, patients' appointments initiated basically a recovery across the pharmaceutical sector and also for our own RUCONEST sale. So the start of the RUCONEST recovery in the UK during Q2 was driven by an increase in new patients because of these doctor appointments and COVID demand. Looking at the revenues in the first half, they went from $97.8 million last year to $93.2 million. And important to note is in Q2 we saw a strong recovery. Revenues increase by 15%, compared to Q2 last year and by 14%, compared to Q1 this year, so by all means, a good Q2 in 2021. Looking at the split, the regional split, the U.S. increased by 60% in the quarter and sales revenues in Europe and the rest of the world decreased from $2 million, almost $2 million to $1.2 million. But that was mainly a result of phasing of ordering. Next slide please. Looking at the gross profit that developed roughly in line with the revenue developments, in fact, the gross margin improved slightly in the first half of the year. And the operating profit decreased in the first half of the year, and also in Q2. And the reason for that is that our operating costs increased from $52 million to $68 million the first half of the year, so an increase of $16 million. And why was that? Well, we continue to invest in the future growth of Pharming. And if you look at the details on the slide, you…

Thank you very much, Jeroen. And please next slide. So, if I look at the investment summary here. We're well funded business. We’re supported by commercial sales and a growing pipeline for the treatment of rare diseases and other medical needs. We’re funded really, as you heard from Jeroen, by the sales of RUCONEST and we brought an another $93 million a Dodge of sales in the first half this year. We have a potential near term inflection point with the 2022 launch of Leniolisib for APDS for treating retargeting new large indications for RUCONEST, recombinant human esterase Phase 2 studies. We also strengthened our early stage pipeline, with the in-licence, potentially curative gene therapy treatment HSC and we have our own transgenic platform candidate for pompous disease in the pipeline. And we're able to leverage commercial infrastructure for in licence for an expanding manufacturing capacity to support the continued RUCONEST demand and C1 inhibitor inhibitor pipeline. And after release, we have an experienced leadership team and board and a very strong balance sheet, both in terms of cash generative potential, and of course, the cash that we have on hand to support our ambitious growth strategy, including potential M&A. And of course, we have this secondary listing on the NASDAQ to be able to finance such transactions in the future, and we are very keen on this and we're very focused on getting transaction -- getting another transaction with the late stage asset in. So next slide, please. Last slide, I will share with you the outlook for the remainder of 2021. So for the remainder, we expect a continued increase in revenues from the sales of RUCONEST, because the pharmaceutical market looks to be normalized -- continue to normalize further and return hopefully to its pre-COVID-19 state. Although, we will continue to monitor the situation and continue to expect some periodic market disruptions. We, however, are maintaining positive earnings during the year. And as Jeroen was explaining, we will continue to invest in the expansion of production facilities for RUCONEST and Leniolisib and the launch -- critical medical affairs and pre-marketing activities for Leniolisib and the registration-enabling study for APDS, as well as the ongoing clinical trials and other development activities including OTL-105. And last but not least, we will look to invest in acquisitions and in licensing of new development opportunities that are near term to the market. So we can launch additional products very rapidly beyond Leniolisib. And we provide no further specific guidance for 2021. So this completes our presentation. I would like now to hand over back to the operator for any questions that you may have that we can answer. Thank you.

Thank you. [Operator Instructions] We have a question from Joe Pantginis from Wainwright. Please go ahead, Joe. Your line is now open.

Hey, guys. Thanks for taking the question.

Hi.

Nice to see the first half results. So a couple of my questions are based on essentially blocking and tackling of the underlying business. So first, can you give us any sort of visibility as to potential next EU territories that might be coming online? Obviously, since you've mentioned for quite some time now that the different regulatory structures there are the rate limiting factor?

Yes. Yes, Joe. Spain, now, we -- after a long period, we got reimbursement. We’re working on Italy as well to get reimbursement. And we're looking at some of the smaller markets as well. I think North Macedonia and Ireland are territories that come to mind. But we're also looking to get reimbursement. And it's not necessarily the regulatory activities, because that, they’re of course -- is of course, approved by EMA as it is by FDA. But in Europe, there’s market access hurdles that, in some cases, are tremendous. And take a long, long time. And that's what you're looking at here. And that's what you're looking at in markets like Spain and Italy and the markets I just mentioned as well.

No, that makes sense. Yes. Sorry.

No. No worries.

Okay. So -- and then just curious, because as the business continues to expand and we'll just ignore COVID right now, that would be great. How are your manufacturing initiatives going? What do you consider to be any rate limiting steps? And then, I guess, I will bring COVID back for my last question about, do you have any potential projected timelines with regard to the COVID data?

Yes, yes. So the first one, we're -- we're executing on the manufacturing expansion strategy, the third facility to produce milk is now nearing its completion. So will soon be populated with those rabbits. And from there on, of course, we will have to generate products from that facility, and process the products all to the finished product and show that to the regulators that we make exactly the same product, which we have done before, of course, so that should not be an issue, but it takes time. But it is in progress. And Pants, we're working on the DSP facility to the purification plant, according to plan, which, of course in a couple of years will come online as well. So we're really insourcing the manufacturing process into the company, and we're executing against it according to plan. With regards to, to the COVID results, we do expect that we could look for some interim analysis results, by the end of Q3 also because our recruitment has significantly slowed down in the studies by now. And that's a good sign, of course, because we want we all want to go back to normalcy. And I think that's what you should be looking at towards the end of the quarter, I would say, we will be coming with some, some updates on that, and some potential next steps. So following these, these two -- these two studies and of course, you were already mentioning it COVID is by far, the normally I mean 50% of the doctors, practices in the US are still not interacting life with pharmaceutical companies. So we're still suffering from this inability to now face to face contact this which of course, is very important when you're dealing with rare diseases, with patients transferring from one to another product in rare diseases situation where you have to go to incredible big, administrative hurdles. And of course, I can fully understand that even when these doctor's offices are open, that may not be their first priority. So that's really an ongoing challenge with PCI turning slowly back to normality and US and therefore, we're optimistic for the rest of the year that, the increase in revenues that we see, should continue for the remainder of the year. Okay.

Thank you, Sijmen.

Okay.

We have another question from Alex Cogut from Kempen. Please go ahead, Alex.

Hi, thanks for taking my questions. I have a couple of questions in pipeline so on RUCONEST, so thanks for kind of describing the dynamics in patients and where you see RUCONEST playing in. But I would like to understand a little bit better. What's actually happening with volume assuming, patients are – you’re getting new patients are using RUCONEST mostly to take HAE attacks. But losing patients that were using a kind of prophylactically off label, aren't you overall experiencing them lower volume of drugs?

Yeah, sometimes we do. Sometimes we don't. It's very difficult, Alex. You're right, there's a mechanism like that. There's also a mechanism that we see that there's a certain saturation point now reached of patient switching over to these new prophylactic therapies that at least will be taught to KOLs, what they tell us. And that means that indeed, you have to replace if you have a severely affected patient, and they go on prophylaxis, they often mostly continue to use RUCONEST but on a lower frequency. That's correct. And if you replace them with patients that are using root canals for breakthrough attacks, you need to replace them with more patients. That is correct. But the good news is, it is happening and the good news is that you can see that we're recovering the sales from where we were hit really hit by COVID, but to more normal levels and we remain optimistic going forward. The challenge here, in addition to that, of course, is face-to-face contact with these doctors, because it is as I already was saying, it's an extensive administrative hurdle to do this. And our experts, internal experts are helping those doctors practices jumping through all these hoops, and sometimes these hoops are very significant before patients actually get their commercial -- their first the delivery of products. However, when patients subscribe to RUCONEST solutions, they get a bridging medication until such time that they are that are reimbursed. So, that's -- that is basically the mechanics that are ongoing in the market. And you see we remain optimistic about the future prospects of RUCONEST in this respect that certainly has its place, and will continue to have its place in this market with this very unpredictable disease.

Got it. And just to clarify the visibility into the volumes per patient, that there are over the last first half?

Yes, we do have insights and that is, of course, all anonymized data. But it is because it's your we working with the specialty pharmacies, we know the patient journeys, and we support patients as well, of course, because these patients are often very much in need of support in all sorts of ways. And we do support them in all sorts of ways. So, yes, we're very close to our patients.

Okay. Got it. On just a question on your qualitative guidance, when you say increased continuous growth in sales, do mean year-over-year or quarter-over-quarter, how should we think about that?

That's always a very nice question, of course. I would say that we started with -- that we increased quarter-on-quarter. I can't speculate on that is really -- that the sales will be higher as last year, because that is certainly very much dependent on how quickly situations turn to normal. And as you know with all pharmaceutical companies are suffering from this issue, that a lot of the CARE is not being delivered, and they suffer from lower revenues, because CARE focused on COVID, takes away resources from CARE that is given otherwise, and I think we're in the same boat here. So, it really depends on the situation returning to normality. And you know what, last year, we lost sales that we didn't get back because our growth was lower than we expected. On the other hand, we got a lot more sales in the end of the year, especially the fourth quarter that we weren't expecting and which turned out to be a certain amount of hoarding by patients, because of that tremendous COVID wave that hit the United States at the end of last year. And that was not foreseen, and you saw the drawback of that. So, it's very difficult to predict that in these kinds of circumstances. But I think it's generally -- you see a general trend of that happening in the pharmaceutical markets. So, we're no exception that.

Okay. Understood. On the pipeline, so when do you expect to report the Leniolisib in the results then?

That's a study that's done by Novartis. So, it's either the end of the year or the beginning of 2022 -- of next year that this comes out -- that the final report is coming out. And we're aiming to bring in the results very quickly to the FDA and EMA and we're aiming for PDUFA date in Q4 of 2022. So you can see that it should be somewhere in the beginning of the year or maybe even towards the end of the year. That's very difficult to predict. As I said, it's in the hands of Novartis. The details. Yeah.

Got it. And on OTL-105, when do you expect to enter the clinic?

Yeah, that's an interesting question. We're doing a lot of preclinical work now. Now with gene therapy as you know your clinical pathway is slightly different because you don't do healthy volunteers. So you go straight into patients here. So we expect somewhat longer preclinical trajectory. And I would like to reserve the answer on that until we have some more insights in that. And we will come out with some more timelines, but it will of course be a few years from now before we go into clinic, but then with the shorter and typical clinical development program, because it's gene therapy. Okay.

Okay. Understood. Thank you very much. That's it.

Thank you, Alex.

We have one more question from Christian Glennie from Stifel. Please go ahead, Christian.

Hi. Thanks for taking the questions. Follow-up then on Leniolisib. Firstly, on -- in terms of who's responsible for the regulatory filing? Is that down to the Novartis or you guys? And then just on the -- think about the tests and that collaboration? Have you thought that yet, is there any information has come out of that, that might inform sort of diagnosis rates? You say, obviously, there's a patient population out there, but it's hard to find them and get the diagnosis, right. So is there anything to come out of that particular initiative as yet?

Yeah. Okay. So first of all, we do the regulatory filing for the product. And then with regards to that APDS testing program with Invitae and navigateAPDS, that's just started, as announced earlier. And we have patients in the market now testing those immunologists there -- immunology practices, and going through those patients and defining the most likely symptom combinations for APDS and educating those immunologists on that and then they offer the genetic testing. And that is actually ongoing, so we have had a number of these tests already being commissioned. We also have a patient registry in Europe and we're building a patient registry in the US. And we have of course, patients identified there. So we start in all owners to build that registry and to build relationships in the community and with the patient organization. There's no specific APDS based organization. There is of course for these primary immune deficiencies. There are several patient organizations where we are collaborating together with. And there is a very high motivation of these patients to actually collaborate with us because of course, if you have an primary immune deficiency and if you happen to have APDS, in this case, you're lucky because you could get some therapy into the future as they are very motivated to work with us. So we look forward to that continuing and of course, in due time, we will inform about where this is going.

Do you have a rough sort of number at this point or any insight into what the diagnosis rate is at the moment for this condition?

Oh, yeah, there are several hundreds of patients already diagnosed for the disease. There's definitely -- we're not starting from scratch at all. We estimate according to the -- estimated prevalence that there could be about 13 out of 50 patients in the market -- in the main markets. And there's a couple of 100 already identified on both sides of the ocean, so we're not starting from scratch at all. Okay, okay.

Okay. And then, just turn to the AKI trial that's underway looking through 200 odd patients, anything to say in terms of recruitment so far in that trial and potential timings of data?

I would say that, I think we're earlier we said that, towards the end of next year, second half of next year, we expect to be able to complete that trial. For now, I would like to stick to that. We're trying to accelerate this study. And again, we will of course, if we're in the market when we are finished the recruitment as per usual. And I think, we should wait for that.

Thank you. And then Suhas [ph] who was asking, the key part, one of the key strategies on business development, something coming on terms of adding new products then to the platform, can you characterize anything versus what you might have said last time in terms of habit, what level of discussions are happening and expectations?

Yeah, we have a very active business development Group. And we are interacting with several potential targets or partners at the same time. And these discussions sometimes go very far and sometimes, we, we stopped very early. So there's a continuous activity going on. But as you know, business development is a real numbers game. And we, of course, are quite precise about the opportunity we're looking for. We're looking for compounds that are in a rare or ultra rare space, because the development is always seeable. We're looking for compounds that are sort of within the window of three years from now to be able to launch. So, if we have a rapid succession of launches following the LENIOLISIB and sia, it has to be of course, we have to be convinced about the data. And then of course, last but not least, the other party will have to dance as well. And that is of course, takes too. So all in all, we're working very hard on this, and we're quite keen that we have continued discussions. But as you know, business development is all nothing until you have a deal, you don't have anything. And that's, we're no exception here, I'm afraid.

So, okay, thank you.

Thank you, Christian.

We do not have any further questions at this stage. I’ll pass it back to Sijmen.

Thank you. Thank you, very much. And thank you very much, ladies and gentlemen. I would like to remind you that we are optimistic about the rest of the year. We are well for the business. We have our own commercial sales. We have a near term inflection points. We're looking at large indications for our lead compounds. We have recently added a very interesting gene therapy to our long term pipeline. And we are looking forward to meet you again with our next results conference. Thank you, very much for your attendance. Goodbye.