Diagnosis: Amyloidosis, Type AL

Thioflavin T fluorochrome dye stain - 4+ positive. 
Congo red positive
Anti-Lambda much stronger immunohistochemical staining than Anti-kappa
Anti-AA immunoperoxidase - negative
Anti-prealbumin (transthyretin) - negative

Additional studies and followup:

Urine for Bence Jones protein revealed excretion of monoclonal free
lambda light chain.

Bone marrow: slight plasmacytosis 4% (normal 0-2%). 

Amyloid detected in periosteum of bone marrow biopsy.

Labial salivary gland biopsy (performed at same time as subcutaneous
biopsy): positive for amyloid in intraglandular vessel walls.

Skeletal survey: No lytic lesions identified.

Serum Beta-2 microglobulin negative.

Management: Symptomatic treatment of cardiomyopathy and continued 
close follow-up with consideration of use of Mephalan and Prednisone.
Patient is age 73.

Determination of amyloid subtype has become essential because of 
prognostic and therapeutic implications which can include cytotoxic
chemotherapy with or without peripheral stem cell bone marrow
transplant and/or heart or liver transplantation depending on the
amyloid subtype.

See excellent comprehensive review and tutorial from Boston University
amyloidosis treatment center engaged in the treatment of
over 200 patients per year with amyloidosis in: 

N Engl J Med 1997 Sep 25;337(13):898-909 
The systemic amyloidoses.
Falk RH, Comenzo RL, Skinner M

Links to PubMed for references

The Systemic Amyloidoses: Current, comprehensive, excellent tutorial. (No abstract)
Senile cardiac amyloidosis: A different pathogenesis than AL amyloidosis
Subcutaneous biopsy for amyloidosis:
Labial salivary gland biopsy for amyloidosis:


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