Study in Subjects With Light Chain (AL) Amyloidosis

Global, multicenter, Phase 2b, open-label extension study of subjects with AL amyloidosis who
had a hematologic response to first-line treatment for their amyloidosis (e.g., chemotherapy,
autologous stem cell transplant [ASCT]) and completed Study NEOD001-201. Subjects in this
study may receive concomitant chemotherapy. Subject screening will occur during the 28 days
prior to the first administration of study drug, which may overlap with the last visit in
Study NEOD001-201. If all eligibility requirements are met, the subject will be enrolled and
Screening assessments will be completed. Study visits will occur every 28 days based on
scheduling from Month 1 Day 1. A ±5-day window is allowed for visits starting after Month 1.
Subjects who discontinue study drug before the End of Study Visit (EOS) should have an Early
Treatment Discontinuation Visit 30 (±5) days after their final administration of study drug.
Each subject's study participation may be up to 38 months or until the study is terminated,
whichever occurs first. The study consists of a Screening Phase (1 month), Treatment Phase
(36 months), and EOS Visit (30 [±5] days after the last dose).

Inclusion Criteria:

1. Completed the End of Study Visit in Study NEOD001-201

2. Adequate bone marrow reserve, hepatic and renal function, as demonstrated by:

- Absolute neutrophil count (ANC) ≥1.0 × 109/L

- Platelet count ≥75 × 109/L

- Hemoglobin ≥9 g/dL

- Total bilirubin ≤2 × upper limit of normal (ULN)

- Aspartate aminotransferase (AST) ≤3 × ULN

- Alanine aminotransferase (ALT) ≤3 × ULN

- Alkaline phosphatase (ALP) ≤5 × ULN (except for subjects with hepatomegaly and
isozymes specific to liver, rather than bone)

- Estimated glomerular filtration rate (eGFR) ≥25 mL/min/1.73 m2 as estimated by
the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation, or
measured GFR ≥25 mL/min/1.73 m2

3. Systolic blood pressure 80-180 mmHg

4. Women of childbearing potential must have a negative pregnancy test during Screening
and must agree to use highly effective physician-approved contraception from Screening
to 90 days following the last study drug administration

5. Male subjects must be surgically sterile or must agree to use highly effective
physician-approved contraception from Screening to 90 days following the last study
drug administration

6. Ability to understand and willingness to sign an informed consent form prior to
initiation of any study procedures

Exclusion Criteria:

1. Any new medical contraindication or clinically significant abnormality on physical,
neurological, laboratory, vital signs, or electrocardiographic (ECG) examination
(e.g., atrial fibrillation; with the exception of subjects for whom the ventricular
rate is controlled) that precludes continuation or initiation of treatment with
NEOD001 or participation in the study

2. Symptomatic orthostatic hypotension that in the medical judgment of the Investigator
would interfere with subject's ability to safely receive treatment or complete study
assessments

3. Myocardial infarction, uncontrolled angina, uncontrolled ventricular arrhythmias, or
ECG evidence of acute ischemia, within 6 months prior to the Month 1-Day 1 Visit

4. Severe valvular stenosis (e.g., aortic or mitral stenosis with a valve area <1.0 cm2)
or severe congenital heart disease

5. ECG evidence of acute ischemia or active conduction system abnormalities with the
exception of any of the following:

- First degree atrioventricular (AV) block

- Second degree AV block Type 1 (Mobitz Type 1/ Wenckebach type)

- Right or left bundle branch block

- Atrial fibrillation with a controlled ventricular rate (uncontrolled [i.e., >110
bpm] ventricular rate is not allowed [determined by an average of three beats in
Lead II or 3 representative beats if Lead II is not representative of the overall
ECG])

6. Has not recovered (i.e., equivalent to a Common Terminology Criteria for Adverse
Events [CTCAE] ≥Grade 2) from the clinically significant toxic effects of prior
anticancer therapy. Exception: subjects who have received treatment with a proteasome
inhibitor such as bortezomib may have CTCAE Grade 2 neuropathy.

7. Received any of the following within the specified time frame prior to the Month 1-Day
1 Visit:

- Oral or IV antibiotics, antifungals, or antivirals within 1 week, with the
exception of prophylactic oral agents. Note: In the event that a subject requires
the chronic use of antivirals, Medical Monitor permission is required for entry
into the study.

- Hematopoietic growth factors, transfusions of blood or blood products within 1
week

- Chemotherapy, radiotherapy, HDAC inhibitors, or other plasma cell directed
therapy within 2 weeks

- ASCT within 4 weeks (i.e., ASCT is allowed if it occurred before enrollment in
Study NEOD001-201 or after completion of Study NEOD001-201 if it was at least 4
weeks before Month 1-Day 1 of this study)

- Major surgery within 4 weeks (or within 2 weeks following consultation with and
approval of Medical Monitor)

- Planned organ transplant during the study

- Any investigational agent, other than NEOD001, within 4 weeks

- Any experimental imaging agent directed at amyloid within 2 weeks

8. Active malignancy with the exception of any of the following:

- Adequately treated basal cell carcinoma, squamous cell carcinoma, or in situ
cervical cancer

- Adequately treated Stage I cancer from which the subject is currently in
remission and has been in remission for ≥2 years

- Low-risk prostate cancer with Gleason score <7 and prostate-specific antigen <10
mg/mL

- Any other cancer from which the subject has been disease-free for ≥2 years

9. History of Grade ≥3 infusion-related adverse events (AEs) or hypersensitivity to
NEOD001

10. History of severe allergy to any of the components of NEOD001 such as
histidine/L-Histidine, Trehalose, or Polysorbate 20

11. Currently known uncontrolled bacterial, viral, fungal, HIV, hepatitis B, or hepatitis
C infection

12. Women who are breastfeeding

13. Any condition which could interfere with, or the treatment for which might interfere
with, the conduct of the study or which would, in the opinion of the Investigator,
unacceptably increase the subject's risk by participating in the study

14. Unable or unwilling to adhere to the study-specified procedures and restrictions

15. Subject is under legal custodianship