Melphalan, Bortezomib, and Stem Cell Transplant in Treating Patients With Primary Systemic Amyloidosis

Inclusion criteria:

DISEASE CHARACTERISTICS:

• Histologically confirmed primary systemic amyloidosis based on the following criteria:

  • Amyloid light-chain disease
  • Deposition of amyloid material by congo red stain showing characteristic green birefringence
  • Monoclonal light chain protein (Bence Jones protein) in the serum or urine, immunohistochemical studies, or serum free light chain assay
  • Evidence of tissue involvement other than carpal tunnel syndrome (i.e., positive immunohistochemical staining of bone marrow demonstrating clonal plasma cells); tissue amyloid deposits with anti-kappa or anti-lambda anti-serum; evidence for a plasma cell dyscrasia by serum/urine or bone marrow; or overwhelmingly convincing clinical features (e.g., macroglossia) associated with other systemic manifestations

PATIENT CHARACTERISTICS:

• Southwest Oncology Group performance status 0-1
• Fertile patients must use effective contraception
• Left ventricular ejection fraction ≥ 45% by Echocardiogram within the past 60 days
• diffusion capacity of lung for carbon monoxide ≥ 50%

PRIOR CONCURRENT THERAPY:

• Prior chemotherapy with alkylating agent allowed provided there is no morphological or cytogenetic evidence of myelodysplastic syndromes
• Prior total cumulative dose of oral melphalan < 300 mg
• At least 4 weeks since prior cytotoxic therapy and fully recovered

Exclusion criteria:

• No senile, secondary, localized, dialysis-related, or familial amyloidosis
• No overt multiple myeloma (> 30% of bone marrow plasmacytosis, extensive [> 2] lytic lesions, or hypercalcemia)
• Not pregnant or nursing
• No myocardial infarction within the past 6 months, congestive heart failure, or arrhythmia refractory to therapy

• No prior malignancy except for any of the following:

  • Adequately treated basal cell or squamous cell skin cancer
  • In situ cervical cancer
  • Adequately treated stage I or II cancer currently in complete remission
  • Any cancer from which the patient has been disease-free ≥ 5 years
• No advanced (grade 3-4) pre-existing neuropathy
• No HIV positivity