V. Perfetti et al., REVERSAL OF NEPHROTIC SYNDROME DUE TO REACTIVE AMYLOIDOSIS (AA-TYPE) AFTER EXCISION OF LOCALIZED CASTLEMANS DISEASE, American journal of hematology, 46(3), 1994, pp. 189-193
The patient (TAL), a chronic asymptomatic HBV carrier with HBsAg-anti-
HBsAg circulating immune complexes, was admitted to our hospital becau
se of a nephrotic syndrome due to renal amyloidosis. There was no fami
ly history of hereditary amyloidosis. Recurrent arthralgias, asthenia,
and weight loss were the prominent clinical features. Laboratory test
results showing that severe chronic inflammatory activity had been pr
esent for 6 years. Interleukin-6 (IL-6) serum concentration was 10 tim
es normal and C-reactive protein was 1.9 mg/ml. A complex immunologica
l picture was also present (immune complex formation, exuberant B cell
reactivity, and decrease in the number of CD4 T cells). A localized f
orm of Castleman's disease (CD) (plasma-cell type) was diagnosed by su
rgical excision of a giant axillary lymph node. AA amyloid was present
in the blood vessels. Within 60 days after excision of the mass, the
systemic symptoms subsided, laboratory signs of inflammatory activity
disappeared and IL-6 serum concentration returned to normal, thus esta
blishing a causal relationship between the localized Castleman's disea
se, elevated IL-6 concentration and the chronic inflammation responsib
le for AA amyloidosis. At 10 months of follow-up, the nephrotic syndro
me has reversed, kidney function has slowly ameliorated, and the patie
nt has gained 12 kg. Abdominal fat aspirates drawn to search for amylo
id, positive before surgery, were subsequently negative. The latter fi
nding, and the remission of the nephrotic syndrome, provided strong ev
idence for regression of the amyloid deposits. However, the HBsAg-anti
-HBsAg immune complexes and depression of T-helper cell activity persi
st. This immunological derangement is therefore not a consequence of C
D. Chronic stimulation of the immune system due to the patient's inabi
lity to eliminate HBV, in the contest of perturbed immunity, may have
favored the genesis of the lymphadenopathy. (C) 1994 Wiley-Liss, Inc.