S0629, Observational Study of Asymptomatic Waldenstrom's Macroglobulinemia and Phase II Study of Tandem Autologous Transplant and Maintenance Treatment for Patients With Symptomatic Disease

Inclusion Criteria

DISEASE CHARACTERISTICS:

- Diagnosis of Waldenstrom macroglobulinemia (WM)

- Measurable disease as determined by IgM protein quantification

- Must be registered to the treatment portion of the study within 28 days of
experiencing disease-related symptoms* AND must present with ≥ 1 of the following
disease-related symptoms:

- Hemoglobin ≤ 11 g/dL

- Platelet count ≤ 100,000/mm³

- Marked tumor mass, defined as lymphadenopathy > 2 cm, palpable hepatomegaly,
splenomegaly, or significant marrow involvement (> 50%)

- Serum albumin < 2.5 g/dL

- Persistently elevated beta-2-microglobulin > 3.0 mg/L in the absence of renal
impairment or active infections

- Presence of B symptoms (i.e., fever, night sweats, or weight loss of > 10% from
baseline)

- Appearance of new or worsening neuropathy manifested by numbness and tingling or
pain

- Symptomatic cryoglobulinemia (i.e., Raynaud phenomenon, skin ulcers, cold
urticaria, or skin necrosis)

- Symptoms of hyperviscosity, if measured viscosity > 4 cp (i.e., new headaches,
vertigo, ataxia, dizziness with or without evident causes of changes in
funduscopic exam, including retinal vein engorgement, hemorrhages, or exudates)

- NOTE: *Appearance of any of the above symptoms caused by WM with no other obvious
cause is a trigger for treatment initiation. Symptoms need not persist for any
specified time frame.

PATIENT CHARACTERISTICS:

- Zubrod performance status 0-2 (Zubrod performance status 3 allowed provided it is
based solely on morbidity due to WM)

- ANC > 1,500/mm³ (unless more marked cytopenias can be explained by marked marrow
involvement or autoimmune myelosuppression)

- Serum creatinine < 3 mg/dL

- Creatinine clearance > 30 mL/min

- SGOT/SGPT < 2 times upper limit of normal

- Direct bilirubin < 2.0 mg/dL

- Not pregnant or nursing

- Negative pregnancy test

- Fertile patients must use effective contraception according to the System for
Thalidomide Education and Prescribing Safety (S.T.E.P.S.®) program

- Ejection fraction ≥ 50% by ECHO or MUGA scan

- Patients with evidence of amyloidosis (i.e., periorbital perforation,
proteinuria not attributable to Bence-Jones protein, unexplained arrhythmias,
increased liver function tests, peripheral neuropathy, carpal tunnel syndrome,
and/or macroglossia) must have an ECHO, rather than MUGA, performed to evaluate
for cardiac amyloidosis (septal thickness, diastolic dysfunction, granular
sparkling, or low-voltage QRS complexes)

- No myocardial infarction within the past 6 months

- No unstable angina

- No difficult-to-control congestive heart failure or cardiac arrhythmias

- No uncontrolled hypertension

- No peripheral neuropathy ≥ grade 2

- No history of multi-infarced dementia or multiple strokes

- No known hypersensitivity to boron or mannitol

- No hepatitis B or C positivity

- No HIV positivity

- No other prior malignancy within the past 5 years except for adequately treated basal
cell or squamous cell skin cancer or carcinoma in situ of the cervix

PRIOR CONCURRENT THERAPY:

- At least 28 days since prior chemotherapy and/or radiotherapy and recovered

- No prior bortezomib

- No concurrent glucocorticoids unless used to control autoimmune disease associated
with WM

- Concurrent participation in the Myeloma Specimen Repository study allowed