The patient underwent a kidney biopsy in November 2017 that revealed HSP nephritis (HSPN, ISKD classified as type III, defined as focal hyperplasia and sclerosis), with isolated positive [kappa]-light chain and negative lambda light chain
immunostaining (Figure 1).
The antibody is designed to bind to insoluble light chain
amyloid protein, including both kappa and lambda subtypes.
(NASDAQ: ALXN) and US-based biotechnology company Caelum Biosciences have forged a collaboration to develop CAEL-101 for light chain
amyloidosis, the companies said.
"The idea here is twofold, "we wanted to understand how light chain
amyloidosis works and how the green tea compound affects this specific protein," Bieschke added.
Urine studies are still widely used in the detection and assessment of free light chain
disease, although recent studies support the value of sFLC assays over urine studies in many circumstances.
Caption: Figure 3: Densitometric profile and SDS-PAGE of a pool of 15 myofibrillar extracts obtained from beef, lamb, and chicken samples extracted with denaturing (a) and nondenaturing (b) solutions (1 = myosin heavy chain; 2 = 180-110 kDa; 3 = [alpha]-actinin; 4 = 95-55 kDa; 5 = desmin; 6 = 51-47 kDa; 7 = actin; 8 = 39 kDa; 9 = troponin T; 10 = tropomyosin; 11 = 33-23 kDa; 12 = myosin light chain
1; 13 = 21-18 kDa; 14 = troponin C; 15 = myosin light chain
2; 16 = 14-10 kDa).
Although bone marrow puncture was not performed in our case, no abnormalities in protein electrophoresis of serum and urine or the serum free light chain
ratio were observed; therefore, the involvement of myeloproliferative diseases was excluded.
Treatment of light chain
(AL) amyloidosis with the combination of bortezomib and dexamethasone.
Wechalekar, "Update on treatment of light chain
amyloidosis," Haematologica, vol.
The immunodiagnostic tests recommended by the International Myeloma Working Group (IMWG) for the initial symptomatic screening of patients for suspected monoclonal gammopathy such as multiple myeloma include serum protein electrophoresis (SPEP), serum immunofixation, and serum free light chains
(free kappa and free lambda with ratio).
In order to putatively identify kappa light chain
expressing B cells, ferret PBMC were stained with DyLight 650 conjugated recombinant Protein L (ThermoFisher, Cat # 21189) and anti-CD79[beta] simultaneously with DyLight 488 and biotin conjugated M[alpha]F-Ig mAb.
In reviewing the literature, we found only a few reports on the role of free light chains
in WM [1-3] and it seems that free light chains
are more sensitive than monoclonal IgM in assessing response to therapy and/or progression of disease  and that prognosis may be correlated with free light chain
levels with 60 mg/L favoring good prognosis and >60 mg/L portending a poor prognosis .