• Information
  1. Primary Amyloid Disease
  2. L Chain Deposition Disease
  3. L and H Chain Deposition Disease
  4. Urate Nephropathy

A. Primary Amyloid Disease

1. Amyloid A related protein (chronic inflammatory protein produced by liver)
2. Fibrils deposited
3. Most patients progress to nephrotic syndrome
4. 20% have multiple myeloma

B. L Chain Deposition Disease

1. Punctate appearance of membranes on electron microscopy (EM) and immunofluorescence
2. Most patients present with proteinuria, without frank nephrotic syndrome
3. Patients often have multiple myeloma with Bence-Jones Proteins
4. Bence-Jones proteins are immunoglobulin light chains
5. Patients can also have monoclonal gammopathy of unknown significance

C. L and H Chain Deposition Disease

1. Usually Type I cryoglobulin, IgM macroglobulinemia
2. Majority have a lymphoproliferative diseae (no rheumatoid factor)
3. Most patients present with albuminuria

D. Urate Nephropathy

1. Uric acid production increased and/or excretion decreased
2. Tumor Lysis Syndrome
3. Polycythemia Vera
4. Gout
5. Lymphomas and Leukemias
6. Fanconi Syndrome
7. Unclear whether uric acid deposition actually affects renal function
8. Lead toxicity must be ruled out (can cause urate nephropathy)