0001047469-05-001308 Sample Contracts

INVESTORS' RIGHTS AGREEMENT
Investors' Rights Agreement • January 24th, 2005 • Gentium S.p.A. • New York

THIS INVESTORS' RIGHTS AGREEMENT is made as of the 15th day of October, 2004, by and between Gentium S.p.A., a joint stock company (società per azioni) incorporated and organized under the laws of the Republic of Italy (the "Company"), and the investors listed on Schedule A hereto, each of which is herein referred to as an "Investor."

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SUPPLY AGREEMENT
Supply Agreement • January 24th, 2005 • Gentium S.p.A.

GENTIUM S.p.A., with registered office in I-22079 Villa Guardia (Como), in Piazza XX Settembre, 2, Inland Revenue code 01192270138, in the person of its Chairman of the Board of Directors and Legal Representative Dr. Laura Iris Ferro, (hereinafter referred to as "GENTIUM")

GENTIUM S.P.A. 2004 EQUITY INCENTIVE PLAN
Equity Incentive Plan • January 24th, 2005 • Gentium S.p.A.
CLINICAL TRIAL AGREEMENT
Clinical Trial Agreement • January 24th, 2005 • Gentium S.p.A.

Agreement made this 27th day of December, 1999 ("Effective Date") between Crinos Industria Farmacobiologica S.p.A., a corporation having an office at Piazza XX Settembre 2, 22079 VILLA GUARDIA [Como], Italy ("Company") and Dana-Farber/Partners Cancer Care, Inc., a non-profit corporation having an office at 44 Binney Street, Boston, Massachusetts 02115 ("DFPCC").

PURCHASE AGREEMENT BY AND BETWEEN SIRTON PHARMACEUTICALS S.p.A. AND GENTIUM S.p.A AND AXCAN PHARMA INC. October 9, 2002
Purchase Agreement • January 24th, 2005 • Gentium S.p.A. • Quebec
LICENSE AND SUPPLY AGREEMENT By and Between GENTIUM S.p.A. AND SIGMA-TAU INDUSTRIE FARMACEUTICHE RIUNITE SpA Dated as of December 7th, 2001
License and Supply Agreement • January 24th, 2005 • Gentium S.p.A.

THIS AGREEMENT ("Agreement"), dated as of December 7th, 2001 (the "Effective Date"), by and between Gentium S.p.A, a corporation organized under the laws of Italy and having a principal place of business at Piazza XX Settembre, 2, Villa Guardia (Como), Italy, capital stock Euro 5.000.000.- fully paid-up, registered with the Company register of Como No 29927, Fiscal Code and VAT Code No. 02098100130("Gentium"), and Sigma-Tau Industrie Farmaceutiche Riunite SpA, a corporation organized under the laws of Italy and having its registered offices at Viale Shakespeare 47, 00144 Rome, Italy, capital stock Euro 15.860.000 fully paid-up, registered with the Company register of Rome No 1468/57, Fiscal Code No. 00410650584 and VAT Code No. 00885531004 ("ST") (with each of Gentium and ST referred to herein individually as a "Party" and collectively as the "Parties").

AMENDEMENT No. 2 TO CLINICAL TRIAL AGREEMENT
Clinical Trial Agreement • January 24th, 2005 • Gentium S.p.A.

This AMENDMENT NO. 2 TO CLINICAL TRIAL AGREEMENT (the "Amendment 2") is made and entered into as of the 28th day of January, 2004, by and between Gentium S.p.A. (formerly Crinos Industria Farmacobiologica S.p.A.) a corporation having an office at Piazza XX Settembre 2, 22079 Villa Guradia, Como Italy (the "Company") and Dana-Farber/Partners Cancer Care, Inc., a non-profit corporation having an office at 44 Binney Street, Boston, Massachusetts, 02115, USA ("DFPCC").

ACTIVE INGREDIENT SUPPLY AGREEMENT
Active Ingredient Supply Agreement • January 24th, 2005 • Gentium S.p.A.

SIRTON PHARMACEUTICALS S.p.A, with registered office in Villa Guardia (COMO), Piazza XX Settembre, 2, Inland Revenue code 01192270138, in the person of Dr. Sauro Carsana, Director (hereinafter referred to as SIRTON)

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Acknowledgement of Sale • January 24th, 2005 • Gentium S.p.A.
AMENDMENT NO. 1 TO CLINICAL TRIAL AGREEMENT
Clinical Trial Agreement • January 24th, 2005 • Gentium S.p.A.

This AMENDMENT NO. I TO CLINICAL TRIAL AGREEMENT (the "Amendment") is made and entered into as of the 19 day of October 2000, by and between Crinos Industria Farmacobiologica S.p.A., a corporation having an office at Piazza XX Settembre 2, 22079 VILLA GUARDIA [Como], Italy (the "Company") and Dana-Farber/Partners Cancer Care, Inc., a non-profit corporation having an office at 44 Binney Street, Boston, Massachusetts 02115 ("DFPCC").

LICENCE AND SUPPLY AGREEMENT between
Licence and Supply Agreement • January 24th, 2005 • Gentium S.p.A.

(Fiscal Code and Vat Code No. 02098100130), with legal offices in Italy at Plazza XX Settembre, no2, 22079 Villa Guardia (CO), acting through its Legal Representative Ms. Laura Iris Ferro, born in Milan on August 3rd, 1951, and domiciled for his office for the purposes of this agreement care of the offices of the Company (hereinafter referred to as "GENTIUM")

FINANCE AGREEMENT ITALIAN REPUBLIC
Finance Agreement • January 24th, 2005 • Gentium S.p.A.

Before me, Dr. Giorgio Miserocchi, notary public in Como, on the Como and Lecce Joint Register of Notaries Public, without the aid of witnesses, waived with my consent, the following parties:

COMMERCIAL LEASE CONTRACT dated January 2, 2001
Commercial Lease Contract • January 24th, 2005 • Gentium S.p.A.

domiciled at the premises forming the subject of the lease, the building in Villa Guardia (COMO) Piazza XX Settembre, 2 under the following terms and conditions:

AGREEMENT FOR THE SUPPLY OF SERVICES
Agreement for the Supply of Services • January 24th, 2005 • Gentium S.p.A.

FINANZIARIA SIRTON S.p.A., with registered office in Villa Guardia (Como), Piazza XX Settembre 2, Inland Revenue code and VAT no. 01192270138, (hereinafter referred to as "FINSIRTON") in the person of Dr. Laura Iris Ferro, Chairwoman

AGREEMENT FOR THE SUPPLY OF SERVICES
Agreement for the Supply of Services • January 24th, 2005 • Gentium S.p.A.

SIRTON PHARMACEUTICALS S.p.A., with registered office in Villa Guardia (COMO), Piazza XX Settembre, 2, Inland Revenue code 01192270138, in the person of Dr. Sauro Carsana, Director (hereinafter referred to as SIRTON);

Agreement between SIRTON PHARMACEUTICALS S.p.A. and GENTIUM S.p.A Relating to the mutual agreement with AXCAN PHARMA INC. Stipulated on 9th October, 2002
Agreement • January 24th, 2005 • Gentium S.p.A.

SIRTON PHARMACEUTICALS S.p.A. (formerly known as CRINOS INDUSTRIA FARMACOBIOLOGICA S.p.A), Piazza XX Settembre 2, 22079 Villa Guardia (Como) (hereinafter referred to as Sirton);

Trial Agreement involving Gentium S.p.A. and the EBMT
Trial Agreement • January 24th, 2005 • Gentium S.p.A.

European Group for Blood and Marrow Transplantation (hereinafter referred to as EBMT), Macdonald Buchanan Building, John Astor House, Foley Street, London W1P 8AN, UK England; and represented by Selim Corbacioglu, Principal Investigator, and Jane Apperley, President of EBMT

SERVICE AGREEMENT
Service Agreement • January 24th, 2005 • Gentium S.p.A.

SIRTON PHARMACEUTICALS S.p.A., with registered office in Villa Guardia (COMO), Piazza XX Settembre, 2, Inland Revenue code 01192270138, in the person of Dr. Sauro Carsana, Director (hereinafter referred to as SIRTON)

Research Agreement between Crinos Industria Farmacobioologica SpA, Villa Guardia (Como) and the Consorzio Mario Negri Sud, Santa Maria Imbaro (Chieti) for the funding of the independent DF-VOD trial: "Defibrotide for the treatment of hepatic...
Research Agreement • January 24th, 2005 • Gentium S.p.A.

The pharmaceutical company Crinos Industria Framacobiologica SpA (hereinafter called "Crinos", having its registered office in Villa Guardia (Como), 22079 Piazza XX Settembre 2, registered at the Chamber of Commerce of Como under no. 172507, Tax Number and VAT no. 01192270138, represented by Dr. Laura Iris Ferro, born in Milan, on 3/8/1951, in her capacity as Deputy Chairperson, domiciled for the position at the registered office of Crinos

SUPPLY AGREEMENT
Supply Agreement • January 24th, 2005 • Gentium S.p.A.

THIS AGREEMENT is made and entered into as of this day of 11th Nov, 2003, by and between SAMIL Pharm. Co., Ltd., a corporation organised and existing under the laws of Korea, with head offices in Seoul, 990-1, Bangbae-Dong, Seocho-Gu ("PURCHASER")

GENTIUM S.p.A. Stock Option Grant Notice (Nonstatutory Stock Option Plan and Agreement)
Stock Option Grant Notice • January 24th, 2005 • Gentium S.p.A. • Texas

Gentium S.p.A., a stock corporation organized under the law of the Republic of Italy, (the "Company"), pursuant to its Nonstatutory Stock Option Plan and Agreement (the "NSO Plan and Agreement") and this Stock Option Grant Notice (the "Grant Notice"), hereby grants to Optionee an option (the "Option") to purchase the number of shares of the Company's common stock set forth below (the "Shares"). This Option is subject to all of the terms and conditions as set forth herein and in the NSO Plan and Agreement, which is attached hereto and incorporated herein in its entirety.

LICENCE AGREEMENT by and between
Licence Agreement • January 24th, 2005 • Gentium S.p.A.

(Fiscal Code and Vat Code No. 02098100130), with legal offices in Piazza XX Settembre, no2, 22079 Villa Guardia (CO), acting through its President and Managing Director Ms. Laura Iris Ferro and domiciled for his office for the purposes of this agreement at the offices of the Company (hereinafter referred to as "GENTIUM")

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UMBRELLA AGREEMENT between
Umbrella Agreement • January 24th, 2005 • Gentium S.p.A.

CRINOS—Industria Farmacobiologica S.p.A., a corporation organised and existing under the law of Italy and having its registered offices in Piazza XX Settembre n. 2,Villa Guardia (CO) Italy, represented by its Vice President and Managing Director Dott.ssa Laura Iris Ferro

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