LCDD is a multisystem disease usually associated with plasma cell dyscrasias or multiple myeloma. It is characterized by the deposition of monoclonal, amorphous, noncongophilic light chains in multiple organs leading to compromised organ function.
Renal involvement is seen very commonly in LCDD, presenting as proteinuria, nephrotic syndrome or renal insufficiency. The liver is usually involved in association with renal lesions. 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.3,4
The current reported patient presented with massive hepatomegaly, severe cholestasis, markedly deranged liver functions and normal renal functions. Cases with large hepatomegaly, disturbance of LFT, cholestasis and portal hypertension have only been reported occasionally.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.1,6
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.1
Liver biopsy in our case showed extensive homogeneous eosinophilic deposits in the lobules and portal areas. The hepatocytes were compressed by the perisinusoidal deposits. Bile plugs were noted in the bile ducts and the canaliculi. However, unlike in the case reported by Faa et al.2
there were no bile infarcts. Cholestasis probably results from the compression of the bile ducts by the portal deposits. Three of the cases with isolated liver involvement previously reported showed deposition of non-amyloid light chains as well as amyloid.2,7,8
Our patient did not show evidence of amyloid deposition.
Leishman stained bone marrow in our patient showed plasmacytosis and extensive deposition of eosinophilic amorphous PAS positive and Congo Red negative "amyloid-like" material. The deposits had a "cumulus cloud" like effect, with an almost transparent center, dense and opaque at the periphery. The presence of light chains in the bone marrow observed in this case is unique and deserves mention. Such deposits have been reported in a patient with amyloidosis.9
Review of the literature shows that this feature has not been described in any of the LCDD cases reported so far.