Isolated liver involvement has been reported so far in only five cases of LCDD; two of these patients were diagnosed to have multiple myeloma and developed LCDD after the onset of treatment.
2,5,6) Four other reported cases of isolated liver LCDD and patients with both liver and renal involvement showed either mild liver enlargement and/only mild to moderate abnormalities in LFT.
Liver involvement in LCDD is usually in the form of deposits over the basement membranes of the biliary tracts and sinusoids, and hepatic parenchyma is usually spared.
9) Review of the literature shows that this feature has not been described in any of the LCDD cases reported so far.
To our knowledge, there are only two other cases in the literature in which LCDD of the liver without renal involvement was the first manifestation of plasma cell dyscrasia.
Lastly, the FLC assay is useful for monitoring disease activity in patients with NSMM, LCMM, AL, and LCDD in whom there is no measurable M-spike on serum or urine protein electrophoresis.
1] Nonstandard abbreviations: FLC, free light chain; NSMM, nonsecretory multiple myeloma; AL, primary systemic amyloidosis; LCDD, light chain deposition disease; LCMM, light chain multiple myeloma; IFE, immunofixation electrophoresis; PCD, plasma cell disorder; MCUS, monoclonal gammopathy of undetermined significance; and SMM, smoldering multiple myeloma.
of cases Multiple myeloma 330 NSMM 20 Osteosclerotic myeloma 15 SMM 72 Indolent/evolving myeloma 8 Plasmacytoma (solitary) 22 Extramedullary myeloma 5 Multiple solitary plasmacytoma 3 Macroglobulinemia 9 IgM lymphoproliferative disease 2 IgM lymphoma 5 Smoldering macroglobulinemia 2 Primary systemic amyloidosis 269 LCDD 7 MGUS 114 Idiopathic Bence Jones proteinuria 4 Heavy chain disease 2 Cryoglobulinemia 4 Acquired Fanconi syndrome 3 Scleromyxedema 2 Plasma cell leukemia 1 Table 2.
The results for 19 LCDD patients are listed in Table 5.
The results from the 25 polyclonal hypergammaglobulinemia sera (Table 3) and 282 reference sera were used to calculate the specificity of FLC K/L, and the results from the 66 patients with AL, MM, or LCDD (Tables 4 and 5) were used to calculate the sensitivity in this selected patient group (Table 6).
Primary systemic amyloidosis and LCDD are often difficult to diagnose, and the presence of a monoclonal FLC is an important differential diagnostic clue.
The FLC data for the 19 LCDD serum samples demonstrate the differences between the FLC and IFE assays.