We retrospectively analyzed clinical presentation, immunopathological data and renal outcome in 13 patients with
glomerulonephritis (GN) and
chronic lymphocytic leukemia (CLL) or related
diffuse well-differentiated lymphocytic lymphoma (WDLL) of B-cell lineage. B-cell proliferation and glomerulopathy were simultaneously diagnosed in seven of the 13 patients.
Nephrotic syndrome was observed in nine patients. Serum
creatinine was elevated (greater than 120 mumol/liter) in 10 patients and exceeded 400 mumol/liter in three patients. A clear cut relationship between GN and
hematologic disease could be established in nine cases: five patients had MPGN caused by type I or type II
cryoglobulinemia; two had MPGN or mesangial
hypertrophy with circulating and deposited noncryoprecipitating monoclonal
IgG K and
IgM K, respectively; in the two remaining patients, monotypic
IgG K glomerular deposits exhibiting fibrillary organization were observed in association with MGN or MPGN, despite the absence of circulating M-component by immunofixation. The pathophysiologic link between glomerular lesions and B-cell proliferation was further evidenced by effectiveness of specific treatment of the
malignancy by
chlorambucil. This
drug, used in the absence of
steroids, induced complete remission of
nephrotic syndrome in the five patients to whom it was given. Moreover, in the five patients with creatininemia greater than 200 mumol/liter who received
chemotherapy, substantial improvement in renal function was observed. These overall data demonstrate that the occurrence of GN in B-CLL and related
lymphoma is not fortuitous, and testify to the paraneoplastic nature of glomerular involvement mediated by deposition and possibly processing of cryoprecipitating or noncryoprecipitating
M-components.