Antibodies are comprised of the structure noted on the left: they have two immunoglobulin heavy chains (in blue) and two immunoglobulin light chains (in green), covalently linked with one another via disulfide bonds (in red). The light chains…
The dilemma: there are a subset of patients with some type of paraproteinemia–e.g., light chain deposition disease or amyloidosis, for instance–which are NEGATIVE by SPEP & UPEP. When there is renal involvement, the diagnosis can potentially be made…
As you may recall, macroglossia (an enlarged tongue) is one of the many manifestations of amyloidosis, the systemic disorder in which a particular protein is deposited as Congo-red positive aggregations. You may also recall that amyloidosis can be…
The finding of fibrils on electron microscopy of a renal biopsy specimen may be an extremely important clue in establishing the diagnosis. In fact, for many of the conditions listed below, the diagnosis may be based PURELY on…
Systemic amyloidosis can involve nearly any organ, and the kidney is often involved. Recall that there are two flavors of amyloidosis: AL amyloidosis (in which the amyloid is formed by light chains as a result of a paraproteinemia)…
The two major forms of amyloidosis are AL amyloidosis (in which light chains form the amyloid deposits) and AA amyloidosis (in which a variety of inflammatory molecules may adopt a beta-pleated structure to deposit as amyloid. A specific…
I saw a patient in Transplant Clinic today with a strong family history of chronic kidney disease requiring dialysis and periodic fevers. She was coming to the Transplant Clinic because she wants to be considered for a potential…
