RATIONALE: Chemotherapy plus interferon alfa may be effective for primary systemic amyloidosis.

PURPOSE: Phase II trial to study the effectiveness of dexamethasone plus interferon alfa in treating patients who have primary systemic amyloidosis.

Condition	Treatment or Intervention	Phase
primary systemic amyloidosis	Drug: dexamethasone  Drug: interferon alfa  Procedure: biological response modifier therapy  Procedure: chemotherapy  Procedure: cytokine therapy  Procedure: interferon therapy	Phase II

Further Study Details: 

OBJECTIVES:

• Evaluate M protein and organ dysfunction responses and overall and progression-free survival in patients with primary systemic amyloidosis treated with dexamethasone/interferon alfa.
• Identify prognostic factors that may relate to response and overall survival in these patients.
• Evaluate the qualitative and quantitative toxic effects of this regimen.

OUTLINE: Patients are stratified by prior amyloidosis treatment (yes vs no).

All patients receive induction therapy with oral dexamethasone on days 1-4, 9-12, and 17-20 every 35 days for a total of 3 courses.

Maintenance therapy begins within 5-8 weeks (within 10 weeks if patients undergo stem cell harvest) of initiation of the third course of induction, as follows: oral dexamethasone for 4 days every 4 weeks; and subcutaneous interferon alfa 3 times per week. Patients who achieved less than a 50% reduction in serum M protein or urinary Bence-Jones protein and who experienced less than grade 3 toxicity during induction receive 3 additional courses of pulse dexamethasone concurrently with entry to maintenance therapy and the initiation of interferon alfa.

Combination therapy is continued until 2 years from entry; thereafter, interferon is administered alone for at least 3 years, toxicity permitting. Patients with stable disease after 5 years of therapy may discontinue interferon alfa at the discretion of the treating physician.

Patients are followed every 6 months for 2 years and yearly thereafter.

PROJECTED ACCRUAL: A total of 100 patients (50 with prior melphalan/prednisone or iododoxorubicin treatment and 50 without) will be entered over 3 years.

Ages Eligible for Study:  18 Years and above,  Genders Eligible for Study:  Both

Criteria

DISEASE CHARACTERISTICS:

• Histologically diagnosed primary systemic amyloidosis based on the following:
• Deposition of fibrillary protein with Congo red positive stain or characteristic electron microscopic appearance
• Monoclonal light chain protein (Bence-Jones protein) in serum or urine or immunohistochemical studies
• Evidence of tissue involvement other than carpal tunnel syndrome
• Diagnostic histologic material available for central pathology review
• Confirmation of tissue diagnosis at all sites of organ dysfunction encouraged
• No senile, secondary, localized, dialysis-related, or familial amyloidosis
• No known therapy-related myelodysplasia

PATIENT CHARACTERISTICS: Age:

• Adult

Performance status:

• SWOG 0-4

Hematopoietic:

• Not specified

Hepatic:

• Not specified

Renal:

• Not specified

Cardiovascular:

• No NYHA class IV status

Other:

• No uncontrolled diabetes
• No active peptic ulcer disease
• No medical condition that precludes high-dose steroids
• No second malignancy within 5 years except:
• Adequately treated nonmelanomatous skin cancer
• In situ cervical cancer
• Adequately treated stage I/II cancer in complete remission
• Not pregnant or nursing
• Effective contraception required of fertile patients
• Blood/body fluid analyses within 14 days prior to registration
• Imaging/exams for tumor measurement within 28 days prior to registration
• Other screening exams within 42 days prior to registration

PRIOR CONCURRENT THERAPY: Biologic therapy

• No prior interferon alfa

Chemotherapy

• Prior melphalan allowed, but recovered from effects
• At least 4 weeks since cytotoxic therapy and recovered

Endocrine therapy

• Prior prednisone allowed, but recovered from effects
• At least 4 weeks since prior glucocorticoids
• No prior dexamethasone
• No planned or concurrent dexamethasone or other therapy for primary systemic amyloidosis

Radiotherapy

• Not specified

Surgery

• Not specified

California
      Rebecca and John Moores UCSD Cancer Center, La Jolla,  California,  92093-0658,  United States
      Veterans Affairs Medical Center - San Francisco, San Francisco,  California,  94121,  United States
Delaware
      CCOP - Christiana Care Health Services, Wilmington,  Delaware,  19899,  United States
District of Columbia
      Lombardi Cancer Center, Washington,  District of Columbia,  20007,  United States
      Walter Reed Army Medical Center, Washington,  District of Columbia,  20307-5000,  United States
Florida
      CCOP - Mount Sinai Medical Center, Miami Beach,  Florida,  33140,  United States
Illinois
      University of Chicago Cancer Research Center, Chicago,  Illinois,  60637-1470,  United States
      Veterans Affairs Medical Center - Chicago (Westside Hospital), Chicago,  Illinois,  60612,  United States
Iowa
      Holden Comprehensive Cancer Center, Iowa City,  Iowa,  52242-1009,  United States
Maryland
      Marlene and Stewart Greenebaum Cancer Center, University of Maryland, Baltimore,  Maryland,  21201,  United States
Massachusetts
      Dana-Farber Cancer Institute, Boston,  Massachusetts,  02115,  United States
      University of Massachusetts Memorial Medical Center - University Campus, Worcester,  Massachusetts,  01655,  United States
Minnesota
      University of Minnesota Cancer Center, Minneapolis,  Minnesota,  55455,  United States
      Veterans Affairs Medical Center - Minneapolis, Minneapolis,  Minnesota,  55417,  United States
Missouri
      Barnes-Jewish Hospital, Saint Louis,  Missouri,  63110,  United States
      Ellis Fischel Cancer Center - Columbia, Columbia,  Missouri,  65203,  United States
      Veterans Affairs Medical Center - Columbia (Truman Memorial), Columbia,  Missouri,  65201,  United States
Nebraska
      University of Nebraska Medical Center, Omaha,  Nebraska,  68198-7680,  United States
Nevada
      CCOP - Southern Nevada Cancer Research Foundation, Las Vegas,  Nevada,  89106,  United States
New Hampshire
      Norris Cotton Cancer Center, Lebanon,  New Hampshire,  03756-0002,  United States
New York
      CCOP - North Shore University Hospital, Manhasset,  New York,  11030,  United States
      CCOP - Syracuse Hematology-Oncology Associates of Central New York, P.C., Syracuse,  New York,  13217,  United States
      Memorial Sloan-Kettering Cancer Center, New York,  New York,  10021,  United States
      Mount Sinai Medical Center, NY, New York,  New York,  10029,  United States
      North Shore University Hospital, Manhasset,  New York,  11030,  United States
      Roswell Park Cancer Institute, Buffalo,  New York,  14263-0001,  United States
      State University of New York - Upstate Medical University, Syracuse,  New York,  13210,  United States
      Veterans Affairs Medical Center - Buffalo, Buffalo,  New York,  14215,  United States
      Veterans Affairs Medical Center - Syracuse, Syracuse,  New York,  13210,  United States
      Weill Medical College of Cornell University, New York,  New York,  10021,  United States
North Carolina
      CCOP - Southeast Cancer Control Consortium, Winston Salem,  North Carolina,  27104-4241,  United States
      Comprehensive Cancer Center at Wake Forest University, Winston Salem,  North Carolina,  27157-1082,  United States
      Duke Comprehensive Cancer Center, Durham,  North Carolina,  27710,  United States
      Lineberger Comprehensive Cancer Center, UNC, Chapel Hill,  North Carolina,  27599-7295,  United States
      Veterans Affairs Medical Center - Durham, Durham,  North Carolina,  27705,  United States
Ohio
      Arthur G. James Cancer Hospital - Ohio State University, Columbus,  Ohio,  43210-1240,  United States
Rhode Island
      Lifespan: The Miriam Hospital, Providence,  Rhode Island,  02906,  United States
Vermont
      Vermont Cancer Center, Burlington,  Vermont,  05401-3498,  United States
      Veterans Affairs Medical Center - White River Junction, White River Junction,  Vermont,  05009,  United States
Virginia
      MBCCOP - Massey Cancer Center, Richmond,  Virginia,  23298-0037,  United States

Study chairs or principal investigators

Laura Fulper Hutchins, MD,  Study Chair,  University of Arkansas   
Richard A. Larson, MD,  Study Chair,  University of Chicago Cancer Research Center   

Study ID Numbers:  CDR0000065092; SWOG-S9628; CLB-9790; CLB-S9628; SWOG-9628
Record last reviewed:  May 2003
Last Updated:  October 13, 2004
Record first received:  November 1, 1999
ClinicalTrials.gov Identifier:  NCT00002849
Health Authority: Unspecified
ClinicalTrials.gov processed this record on 2005-04-08