Press Release: Pharming Reports Second Quarter and First Half 2026 Financial Results and Revises Year-end Guidance; Strong Joenja Momentum and Near-term Clinical Readouts to Support Broader Therapeutic Use

Dow Jones07-30

For investor audiences and media only

   -- Second quarter 2026 total revenues decreased by 3% to US$90.2 million, 
      compared to the second quarter 2025 
 
   -- RUCONEST$(R)$ revenue was US$72.3 million, a 10% decrease compared to the 
      second quarter 2025 and a 24% increase compared to the first quarter 
      2026, with active patient base 93% of year-ago and strong new patient 
      enrollments 
 
   -- Joenja(R) revenue was US$17.9 million, a 40% increase compared to the 
      second quarter 2025, reflecting continued strong U.S. and international 
      growth, with first European launch in Germany after quarter-end 
 
   -- Second quarter operating profit amounted to US$1.3 million compared to 
      US$10.8 million in the second quarter 2025, impacted by 
      manufacturing-related inventory impairments, lower revenue and France 
      site closure 
 
   -- Plans to report clinical data with leniolisib in significantly larger 
      CVID patient populations in the fourth quarter 2026 
 
   -- Updates 2026 total revenue guidance to US$375 million - US$395 million, 
      reflecting a US$30 million reduction, and improves operating expense 
      guidance by US$15 million to US$315 million - US$320 million 
 
   -- Pharming to host a conference call today at 13:30 CEST (7:30 am EDT) 

Leiden, the Netherlands, July 30, 2026: Pharming Group N.V. (Euronext Amsterdam: PHARM/Nasdaq: PHAR) presents its preliminary (unaudited) financial report for the second quarter and first half year ended June 30, 2026.

Chief Executive Officer, Fabrice Chouraqui, commented:

"While we have lowered our full-year revenue guidance, we are encouraged by the resilience of RUCONEST, a year after the launch of the first oral on-demand HAE treatment. The active patient base remained at 93% of year ago levels, and RUCONEST continues to be a cornerstone on-demand treatment for high-burden HAE patients. Supported by strong new patient enrollments in the second quarter, we expect RUCONEST revenues to stabilize and return to growth during the second half of 2026.

Joenja (leniolisib) continued to build momentum in APDS, supported by expansion into additional geographies. Beyond APDS, we are encouraged by the potential for leniolisib in broader populations with primary immunodeficiencies, including CVID, where the PI3K pathway is seen as a key driver of immune dysregulation, and we look forward to reporting data from two clinical trials in the fourth quarter of 2026.

As we continue to implement a more disciplined operating model, reflected in our improved full-year operating expense guidance, Pharming is evolving into a more diversified rare disease company with a high-value pipeline that has the potential to materially increase our scale and strengthen our long-term growth profile."

Second quarter and first half 2026 highlights

Commercialized assets

RUCONEST marketed for the treatment of acute HAE attacks

RUCONEST revenue in the second quarter of 2026 was US$72.3 million, a 10% decrease compared to the second quarter of 2025 and a 24% increase compared to the first quarter of 2026. Revenue for the first half of 2026 was US$130.7 million, a 12% decrease compared to the same period in 2025.

RUCONEST revenue in the current quarter compared to the second quarter of 2025 was impacted by market dynamics in the U.S. (reducing revenue by 6%), inventory drawdowns at U.S. specialty pharmacy customers, and the completion of the planned withdrawal from non-U.S. markets.

With its differentiated efficacy, reliability and rapid onset of action via IV administration, RUCONEST remains a trusted on-demand treatment option for high-burden patients experiencing more severe or frequent attacks who have failed other on-demand medications, despite increasing competition. The overwhelming majority of RUCONEST patients remain on therapy with the active patient base at 93% of year ago levels. Notably, we achieved a significant increase in new patient enrollments and continued to add new prescribers in the current quarter, indicating continued physician confidence and improvement in underlying performance.

Joenja (leniolisib) marketed for the treatment of APDS

Joenja revenue increased to US$17.9 million in the second quarter of 2026, a 40% increase compared to the second quarter of 2025. Revenue for the first half of 2026 was US$32.0 million, a 37% increase compared to the same period in 2025.

Year-over-year revenue growth in the current quarter was driven by a strong increase in patients on paid therapy in the U.S., inventory normalization at U.S. specialty pharmacy customers following greater drawdowns in the first quarter, and increased demand in international markets.

The U.S. market contributed 86% of second quarter revenues, while the EU and Rest of World contributed 14%.

As of June 30, 2026, 132 patients were on paid therapy in the U.S., representing a 16% increase from the 114 patients at the end of the second quarter of 2025 and an increase of 5 patients during the quarter.

APDS patient finding

As of June 30, 2026, we have identified 1,042 diagnosed APDS patients of all ages globally, including 298 patients in the U.S. and 387 in core markets outside of the U.S. Of the identified patients in the U.S., 198 patients are 12 years of age or older and currently eligible for treatment with Joenja, while 60 are between 4 and 11 years of age.

Joenja (leniolisib) development

Leniolisib for APDS

As of June 30, 2026, there are 188 APDS patients in either a leniolisib Expanded Access Program (compassionate use), an ongoing clinical study, or a paid access program.

Pediatric label expansion

On June 4, 2026, we announced that the U.S. Food and Drug Administration (FDA) had accepted our resubmitted supplemental New Drug Application (sNDA) seeking approval for Joenja as a treatment for children aged 4 to 11 years with APDS. Following the Complete Response Letter $(CRL)$ received on January 30, 2026, and a subsequent Type A meeting with the FDA on March 26, 2026, the resubmission seeks approval of 40 mg and 50 mg twice-daily dosing for pediatric patients weighing 27 kg or more, who represent a meaningful proportion of the identified pediatric patient population. The FDA has assigned a Prescription Drug User Fee Act (PDUFA) target action date of October 24, 2026.

We plan to submit a separate sNDA in the coming days, seeking approval for lower doses in patients weighing 13 - 27 kg.

Japan

We expect to launch Joenja for the treatment of APDS in adult and pediatric patients aged 4 years and older in the third quarter of 2026.

European Economic Area $(EEA)$

On May 22, 2026, we announced that the European Commission $(EC)$ had granted Marketing Authorization for Joenja as the first and only approved treatment of APDS in adult and pediatric patients 12 years of age and older. Joenja was commercially launched in Germany on July 1, 2026, marking the first European launch following EC approval, with additional launches anticipated pending completion of national reimbursement negotiations.

Other countries

On July 8, 2026, Joenja was approved by Health Canada for the treatment of APDS in adult and pediatric patients 12 years of age and older.

On July 10, 2026, Joenja was approved by the South Korea Ministry of Food and Drug Safety for the treatment of APDS in adult and pediatric patients 12 years of age and older.

Leniolisib for additional primary immunodeficiencies (PIDs)

Two Phase II clinical trials are evaluating leniolisib for additional primary immunodeficiencies (PIDs) with immune dysregulation, including genetically identifiable PIDs linked to altered PI3K signaling and common variable immunodeficiency or CVID, which represent substantially larger patient populations than APDS. Patient enrollment in both clinical trials is complete and we anticipate trial read-outs in the fourth quarter of 2026, consistent with prior guidance. The PI3K pathway is seen as a key driver of immune dysregulation in many PIDs, and we currently anticipate conducting a single registrational Phase III trial in the broader CVID indication, incorporating patient populations from both studies.

A presentation at the 2026 Annual Meeting of the Clinical Immunology Society (CIS), which took place May 6-9, included clinician expanded access experience with leniolisib to treat immune dysregulation in patients with CVID and CVID-like disorders. Clinician-reported outcomes demonstrated improvements, with no progression, in clinical manifestations of immune dysregulation as well as improvements in patients' quality of life.

Other events

As we continue to improve operating efficiency in line with our strategy, we have decided to close our production-support site in Évry, France, with the closure expected to take effect in the fourth quarter of 2026. As a result, we recognized a one-time charge in the current quarter to reflect the estimated costs associated with the planned closure.

Financial Summary

 
Consolidated Statement of Income          2Q 2026  2Q 2025  1H 2026  1H 2025 
----------------------------------------  -------  -------  -------  ------- 
Amounts in US$m except per share data 
----------------------------------------  -------  -------  -------  ------- 
Total Revenues                               90.2     93.2    162.7    172.3 
----------------------------------------  -------  -------  -------  ------- 
Cost of sales                              (14.5)    (9.0)   (21.2)   (17.3) 
----------------------------------------  -------  -------  -------  ------- 
Gross profit                                 75.7     84.2    141.5    155.0 
----------------------------------------  -------  -------  -------  ------- 
Other income                                  1.4      1.8      1.8      2.2 
----------------------------------------  -------  -------  -------  ------- 
Research and development                   (32.3)   (23.7)   (57.8)   (44.8) 
----------------------------------------  -------  -------  -------  ------- 
General and administrative                 (14.9)   (20.5)   (30.2)   (43.0) 
----------------------------------------  -------  -------  -------  ------- 
Marketing and sales                        (28.6)   (31.0)   (58.9)   (65.6) 
----------------------------------------  -------  -------  -------  ------- 
Other Operating Costs                      (75.8)   (75.2)  (146.9)  (153.4) 
----------------------------------------  -------  -------  -------  ------- 
Operating profit (loss)                       1.3     10.8    (3.6)      3.8 
----------------------------------------  -------  -------  -------  ------- 
Finance result (net) and share of 
 result in associates                         1.8    (3.7)      1.9    (8.5) 
----------------------------------------  -------  -------  -------  ------- 
Profit (loss) before tax                      3.0      7.1    (1.7)    (4.7) 
----------------------------------------  -------  -------  -------  ------- 
Income tax credit (expense)                 (1.4)    (2.5)    (1.9)    (5.6) 
----------------------------------------  -------  -------  -------  ------- 
Profit (loss) for the period                  1.6      4.6    (3.6)   (10.3) 
----------------------------------------  -------  -------  -------  ------- 
Earnings per share 
----------------------------------------  -------  -------  -------  ------- 
Basic, attributable to equity holders 
 of the parent (US$)                        0.003    0.007  (0.005)  (0.015) 
----------------------------------------  -------  -------  -------  ------- 
Diluted, attributable to equity holders 
 of the parent (US$)                        0.002    0.006  (0.005)  (0.015) 
----------------------------------------  -------  -------  -------  ------- 
 
 
Segment information - Revenues    2Q 2026  2Q 2025  1H 2026  1H 2025 
--------------------------------  -------  -------  -------  ------- 
Amounts in US$m 
--------------------------------  -------  -------  -------  ------- 
Revenue - RUCONEST (US)              72.0     79.6    130.2    146.2 
--------------------------------  -------  -------  -------  ------- 
Revenue - RUCONEST (EU and RoW)       0.3      0.8      0.4      2.8 
--------------------------------  -------  -------  -------  ------- 
Total Revenues - RUCONEST            72.3     80.4    130.7    149.0 
--------------------------------  -------  -------  -------  ------- 
Revenue - Joenja (US)                15.4     11.8     26.9     21.3 
--------------------------------  -------  -------  -------  ------- 
Revenue - Joenja (EU and RoW)         2.5      1.0      5.1      2.0 
--------------------------------  -------  -------  -------  ------- 
Total Revenues - Joenja              17.9     12.8     32.0     23.3 
--------------------------------  -------  -------  -------  ------- 
 
Total Revenues - US                  87.4     91.4    157.1    167.5 
--------------------------------  -------  -------  -------  ------- 
Total Revenues - EU and RoW           2.8      1.8      5.5      4.8 
--------------------------------  -------  -------  -------  ------- 
 
Total Revenues                       90.2     93.2    162.7    172.3 
--------------------------------  -------  -------  -------  ------- 
 
 
                                                       December 31, 
Consolidated Balance Sheet              June 30, 2026      2025 
--------------------------------------  -------------  ------------ 
Amounts in US$m 
--------------------------------------  -------------  ------------ 
Cash and cash equivalents, restricted 
 cash and marketable securities                 159.5         181.1 
--------------------------------------  -------------  ------------ 
Current assets                                  280.7         299.5 
--------------------------------------  -------------  ------------ 
Total assets                                    468.0         500.0 
--------------------------------------  -------------  ------------ 
Current liabilities                              93.2         115.8 
--------------------------------------  -------------  ------------ 
Shareholders' equity                            270.5         277.1 
--------------------------------------  -------------  ------------ 
 

Figures may not add up due to rounding.

Financial highlights

Second quarter 2026

For the second quarter of 2026, total revenues decreased by US$3.0 million, or 3%, to US$90.2 million, compared to US$93.2 million in the second quarter of 2025. RUCONEST revenues amounted to US$72.3 million, a 10% decrease compared to the second quarter of 2025. The decrease in RUCONEST(R) revenues was primarily driven by a decrease in volume. Joenja revenues amounted to US$17.9 million in the second quarter of 2026, a 40% increase compared to the second quarter of 2025. This increase in Joenja revenues was primarily driven by an increase in volume.

Gross profit decreased by US$8.5 million, or 10%, to US$75.7 million, compared to US$84.2 million in the second quarter of 2025, mainly due to manufacturing-related impairments of inventory (US$4.9 million) and the decrease in revenues.

The operating profit amounted to US$1.3 million compared to US$10.8 million in the second quarter of 2025. Excluding US$4.9 million in one-time manufacturing-related impairments of inventory and US$1.7 million in expenses associated with the planned closure of the production-support site in Évry, France, adjusted operating profit(1) in the second quarter 2026 amounted to US$7.9 million. Excluding US$2.1 million of non-recurring Abliva acquisition-related expenses, adjusted operating profit in the second quarter 2025 amounted to US$12.9 million. The operating result was primarily impacted by manufacturing-related impairments of inventory and a decrease in revenues, while operating expenses remained similar to the second quarter of 2025.

The finance result (net) and share of result in associates amounted to a gain of US$1.8 million compared to a loss of US$3.7 million in the second quarter of 2025. This improvement was mainly driven by favorable EUR/USD exchange rate movements, resulting in a foreign currency gain of US$2.9 million in 2026, compared to a loss of US$1.9 million in the second quarter of 2025.

The Company had a net profit of US$1.6 million, compared to US$4.6 million in the second quarter of 2025. The effect of the aforementioned drivers was partially offset by a favorable change in the net finance result.

Cash used in operations amounted to US$9.7 million, compared to US$11.7 million cash generated from operations in the second quarter of 2025. Cash and cash equivalents, restricted cash and marketable securities decreased from US$171.8 million at the end of first quarter of 2026 to US$159.5 million at the end of the second quarter of 2026, primarily driven by unfavorable working capital movements, mainly a decrease in trade and other payables and an increase in inventories, as well as income tax payments, partially offset by collections of trade and other receivables.

(1) Adjusted Operating Profit is a non-IFRS measure used by management to assess underlying operating performance and provides additional insight into the Company's core operating profitability. It excludes certain non-core items.

First half year 2026

Total revenues decreased 6% during the first half of 2026 to US$162.7 million, compared to US$172.3 million during the first half of 2025. For the first half of 2026, total RUCONEST revenues were 12% lower at US$130.7 million, compared to revenues of US$149.0 million for the first half of 2025. The decrease in RUCONEST revenues was primarily driven by a decrease in volume.

Joenja revenues amounted to US$32.0 million in the first half of 2026, a 37% increase compared to the first half of 2025. This increase in Joenja revenues was primarily driven by an increase in volume.

Gross profit decreased by US$13.5 million, or 9%, to US$141.5 million, compared to US$155.0 million in the first half of 2025, mainly due to the decrease in revenues and manufacturing-related impairments of inventory (US$4.9 million). Further details on revenue and gross profit segmentation is provided in Note 7. Segment information in the Notes to the condensed consolidated interim financial statements of this press release.

The operating loss amounted to US$3.6 million compared to an operating profit of US$3.8 million in the first half of 2025. Excluding US$4.9 million in one-time manufacturing-related impairments of inventory and US$1.7 million in expenses associated with the planned closure of the production-support site in Évry, France, adjusted operating profit in the first half 2026 amounted to US$3.0 million. Excluding US$9.9 million of non-recurring Abliva acquisition-related expenses, adjusted operating profit in the first half 2025 amounted to US$13.7 million. The deteriorated operating result was primarily driven by a decrease in revenues and manufacturing-related impairments of inventories in 2026.

The finance result (net) and share result in associates amounted to a gain of US$1.9 million compared to a loss of US$8.5 million in the first half of 2025. This improvement was mainly driven by favorable EUR/USD exchange rate movements, resulting in a foreign currency gain of US$5.3 million in the first half year of 2026, compared to a loss of US$4.5 million in the first half year of 2025.

The Company had a net loss of US$3.6 million, compared to a net loss of US$10.3 million in the first half of 2025. In addition to the aforementioned drivers, the net result was positively impacted by a lower tax expense of US$1.9 million compared to US$5.6 million in the first half of 2025.

Cash used in operations amounted to US$7.7 million, compared to US$12.0 million of cash generated from operations in the first half of 2025. Cash and cash equivalents, restricted cash and marketable securities decreased by US$21.6 million to US$159.5 million from US$181.1 million at the end of 2025, primarily driven by negative working capital movements, increased income tax payments and US$12.3 million settlement of the lease liability following the early termination of the DSP facility lease at Pivot Park in Oss, the Netherlands.

Outlook/Summary

For 2026, the Company anticipates:

   -- Total revenues between US$375.0 million and US$395.0 million (0% to 5% 
      growth), updated to reflect a US$30 million reduction compared with prior 
      guidance. 
 
   -- Total operating expenses between US$315.0 million and US$320.0 million 
      (1% to 3% growth), including over US$40 million incremental R&D 
      investment to advance the pipeline and US$9 million structural G&A cost 
      reductions based on the plan announced in October 2025, reflecting an 
      improvement of US$15 million from prior guidance. 
 
   -- RUCONEST revenue stabilization and return to growth during second half of 
      2026, and significant and accelerating annual Joenja U.S. and ex-U.S. 
      growth. 
 
   -- Additional regulatory approvals and commercial launches for leniolisib 
      for APDS patients 12 years of age or older and for pediatric label 
      expansion in key global markets. 
 
   -- Top-line data readouts for the two ongoing leniolisib Phase II clinical 
      trials in PIDs with immune dysregulation, including CVID, to expand the 
      asset's addressable patient population. 
 
   -- Completion of enrollment in the pivotal FALCON clinical study for 
      napazimone (KL1333) in mitochondrial DNA-driven primary mitochondrial 
      diseases. 
 
   -- Enhancing capital efficiency to drive growth and build a leading global 
      rare disease company. 
 
   -- Continued focus on potential acquisitions and in-licensing of clinical 
      stage opportunities in rare diseases. Financing, if required, would come 
      via a combination of our strong balance sheet and access to capital 
      markets. 

No further specific financial guidance for 2026 is provided.

Trademarks

Joenja(R) and RUCONEST(R) are registered trademarks owned by or licensed to Pharming Group N.V. or its affiliates.

Additional information

Presentation

The conference call presentation is available on the Pharming.com website from 07:30 CEST today.

Conference Call

The conference call will begin at 13:30 CEST/07:30 EDT on Thursday, July 30. A transcript will be made available on the Pharming.com website in the days following the call.

Please note, the Company will only take questions from dial-in attendees.

Webcast Link:

https://edge.media-server.com/mmc/p/m35zejc7

Conference call dial-in details:

https://register-conf.media-server.com/register/BId285845cff504c39931913d279f646ea

Additional information on how to register for the conference call/webcast can be found on the

Pharming.com website.

Financial Calendar 2026

3Q 2026 financial results November 5, 2026

For further public information, contact:

Pharming

Michael Levitan, VP Investor Relations and Capital Markets

T: +1 (908) 705 1696

E: investor@pharming.com

Saskia Mehring, Head of Corporate Communications

T: +31 6 28 32 60 41

E: media.relations@pharming.com

Media Relations

Julia Deutsch (Lyra Strategic Advisory on behalf of Pharming)

E: JDeutsch@lyraadvisory.com

Netherlands: Leon Melens (LifeSpring Life Sciences Communication on behalf of Pharming)

T: +31 6 53 81 64 27

About Pharming Group N.V.

Pharming Group N.V. (Euronext Amsterdam: PHARM/Nasdaq: PHAR) is a global biopharmaceutical company dedicated to transforming the lives of patients with rare, debilitating, and life-threatening diseases. We develop and commercialize innovative medicines, including small molecules and biologics. Pharming is headquartered in Leiden, the Netherlands, with U.S. and European operations.

For more information, visit www.pharming.com and find us on LinkedIn.

Auditor's involvement

The Condensed Consolidated Interim Financial Statements have not been audited by the Company's statutory auditor.

Responsibility Statement

The Board of Directors of the Company (the "Board") hereby declares that to the best of its knowledge, the condensed consolidated interim financial statements, which have been prepared in accordance with IAS 34 (interim financial reporting), give a true and fair view of the assets, liabilities, financial position and profit or loss of the Company, and this interim Board report includes a fair review of the information required pursuant to section 5:25d(8) and (9) of the Dutch Financial Supervision Act (Wet op het financieel toezicht).

Leiden, July 30, 2026

Fabrice Chouraqui, Chief Executive Officer and Executive Director

Richard Peters, Non-Executive Director and Chairman of the Board of Directors

Mark Pykett, Non-Executive Director

Barbara Yanni, Non-Executive Director

Leonard Kruimer, Non-Executive Director

Jabine van der Meijs, Non-Executive Director

Elaine Sullivan, Non-Executive Director

Forward-looking Statements

This press release may contain forward-looking statements. Forward-looking statements are statements of future expectations that are based on management's current expectations and assumptions and involve known and unknown risks and uncertainties that could cause actual results, performance, or events to differ materially from those expressed or implied in these statements. These forward-looking statements are identified by their use of terms and phrases such as "aim", "ambition", "anticipate", "believe", "could", "estimate", "expect", "goals", "intend", "may", "milestones", "objectives", "outlook", "plan", "probably", "project", "risks", "schedule", "seek", "should", "target", "will" and similar terms and phrases. Examples of forward-looking statements may include statements with respect to timing and progress of Pharming's preclinical studies and clinical trials of its product candidates, Pharming's clinical and commercial prospects, and Pharming's expectations regarding its projected working capital requirements and cash resources, which statements are subject to a number of risks, uncertainties and assumptions, including, but not limited to the scope, progress and expansion of Pharming's clinical trials and ramifications for the cost thereof; and clinical, scientific, regulatory, commercial, competitive and technical developments. In light of these risks and uncertainties, and other risks and uncertainties that are described in Pharming's 2025 Annual Report and the Annual Report on Form 20-F for the year ended December 31, 2025, filed with the U.S. Securities and Exchange Commission, the events and circumstances discussed in such forward-looking statements may not occur, and Pharming's actual results could differ materially and adversely from those anticipated or implied thereby. All forward-looking statements contained in this press release are expressly qualified in their entirety by the cautionary statements contained or referred to in this section. Readers should not place undue reliance on forward-looking statements. Any forward-looking statements speak only as of the date of this press release and are based on information available to Pharming as of the date of this release. Pharming does not undertake any obligation to publicly update or revise any forward-looking statement as a result of new information, future events or other information.

Inside Information

This press release relates to the disclosure of information that qualifies, or may have qualified, as inside information within the meaning of Article 7(1) of the EU Market Abuse Regulation.

Pharming Group N.V.

Condensed Consolidated Interim Financial Statements in US Dollars (unaudited)

For the period ended June 30, 2026

   -- Condensed consolidated interim statement of income 
 
   -- Condensed consolidated interim statement of comprehensive income 
 
   -- Condensed consolidated interim balance sheet 
 
   -- Condensed consolidated interim statement of changes in equity 
 
   -- Condensed consolidated interim statement of cash flow 
 
CONDENSED CONSOLIDATED INTERIM STATEMENT OF INCOME 
 (UNAUDITED) 
For the period ended June 30 
 
Amounts in US$ '000                         notes   1H 2026    1H 2025 
------------------------------------------  -----  ---------  --------- 
Revenues                                        7    162,660    172,315 
------------------------------------------  -----  ---------  --------- 
Costs of sales                                  9   (21,189)   (17,295) 
------------------------------------------  -----  ---------  --------- 
Gross profit                                    7    141,471    155,020 
------------------------------------------  -----  ---------  --------- 
Other income                                    8      1,801      2,232 
------------------------------------------  -----  ---------  --------- 
Research and development                            (57,819)   (44,837) 
------------------------------------------  -----  ---------  --------- 
General and administrative                          (30,165)   (42,991) 
------------------------------------------  -----  ---------  --------- 
Marketing and sales                                 (58,886)   (65,619) 
------------------------------------------  -----  ---------  --------- 
Other Operating Costs                           9  (146,870)  (153,447) 
------------------------------------------  -----  ---------  --------- 
Operating profit (loss)                              (3,598)      3,805 
------------------------------------------  -----  ---------  --------- 

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