| Clinical Pathologic Conference: Amyloidosis: Complications and Treatment | ||
Feb. 10, 1999 12 noon-1 p.m. Lipsett Amphitheater | ||
Moderator: James E. Balow, MD, Clinical Director and Chief, Kidney Disease Section, NIDDK Speakers: George Csako, MD, Senior Staff Advisor, Clinical Chemistry, CC; Richard Cannon, MD, Acting Chief, Cardiology Branch, NHLBI; Marinos Dalakas, MD, Chief, Neuromuscular Diseases Section, NINDS; John Tisdale, MD, Tenure-track Investigator, Molecular and Clinical Hematology Branch, NIDDK | ||
1. Case presentation: Balow (3 min):
68 yo WM presenting with apparent primary nephrotic syndrome. Initial renal biopsy on outside suggested focal sclerosis. Treated with steroids, cyclosporine, mycophenolate, cyclophosphamide without effect on nephrotic syndrome. Referred to NIH for focal sclerosis protocol. Renal function normal. Amyloid found in kidney and skin. SPEP: nephrotic syndrome pattern with no spikes. UPEP: nephrotic syndrome pattern; IFE: lambda light chain spike hidden in beta region of UPEP.
2. Laboratory detection of paraproteins, including those with amyloidogenic potential: Csako (5 min)
Demonstration of the index patient's serum and urine electrophoresis findings. General overview of use of SPEP, UPEP and immunofixation electrophoresis in proteinuria or in other cases of suspected paraproteinemias. Description of "urinary ladder" pattern. Brief reference to current recommendations on lab diagnosis and follow-up (New Guidelines for Clinical and Laboratory Evaluation of Patients with Monoclonal Gammopathies by CAP Conference XXXII--to be published Spring 1999)
3. Clinicopathologic diagnosis of amyloidosis: Balow (5 min)
- Diagnostic approach to amyloidosis, including roles of various tissue biopsies.
- Overview of the classification of amyloidosis.
4. Renal aspects of amyloidosis: Balow (5 min)
Clinical manifestations and natural history of various forms of renal amyloid (emphasizing AL).
5. Cardiac amyloidosis: Cannon (10 min)
Clinical manifestations and natural history of various forms of cardiac amyloid (emphasizing AL).
6. Amyloid-associated neuropathies: Dalakas (5 min)
Clinical manifestations and natural history of various forms of neuropathic amyloid (emphasizing AL).
7. New approaches to treatment of AL amyloidosis: Tisdale (10 min)
- Relationship of AL amyloid to benign monoclonal gammopathy of unknown significance, other light chain disorders, and multiple myeloma.
- Conventionaltreatment of AL amyloid.
- New approaches with ablative chemotherapy, stem cell and growth factor rescue.
8. Patient outcome and summation: Balow (2 min)
9. Exchanges with audience: (10 min)
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Questions about the Clinical Center or CC grand rounds? OCCC@nih.gov Or call: (301) 496-2563 National Institutes of Health, Warren Grant Magnuson Clinical Center, Bethesda, Maryland 20892 . Last modified 1/99 |