39-year-old man with no toxic habits, allergic to acetylsalicylic acid (ASA) and diclofenac.
She initially consulted for soft tissue swelling associated with palpable purpura in both lower extremities in April 2001.
Immunological study revealed positive cryoglobulinemia with 9.2% cryocrypt (monoclonal kappa IgM and IgG component).
Serology for hepatotropic virus and human immunodeficiency virus (HIV) were negative, as well as negative antiphospholipid antibodies.
Physical examination revealed palpable purpura in the lower limbs; the rest of the examination was anodyne.
A computed tomography (CT) scan revealed adenopathies and visceromegaly.
With the orientation of cutaneous vasculitis, treatment with prednisone at a dose of 1 mg/kg/day was started, with a favorable initial evolution and disappearance of the lesions.
One year after the first consultation, the patient complained of paresthesia in the lower limbs, again associated with petechiae in that location, presenting nephritic syndrome.
Laboratory tests showed hypocomplementemia, 17% cryocrypt and proteinogram with weak anomalous band in the gamma area.
Serum immunoelectrophoresis: restricted mobility component IgM kappa (in urine there was no suspect element of monoclonality).
The renal function showed a creatinine level of 1.2 mg/dl, sediment with seizures +++ and proteinuria of 2.8 g/day.
Renal biopsy confirmed the presence of glomerulonephritis with mesangiocapilary pattern.
Treatment was established with azathioprine, maintaining renal function and proteinuria around 1 g.
1.
After six years of follow-up, an analytical control showed immunophenotype of peripheral blood compatible with LLP.
This finding was later confirmed with a bone marrow aspirate in which it was observed that in chronic lymphoproliferative syndrome of small cell line B, compatible with quiescent LLP treatment, this time was not due to chemotherapy.
A year later the patient had persistent proteinuria of 4.7 g/24 h, so it was decided to perform a new renal biopsy that confirmed the presence of a glomerulonephritis cryoglobulinemic lymphocytic involvement and also showed low grade lymphocytic lymphoma.
Because of the renal involvement caused by lymphoma, it was decided to start treatment with rituximab at a dose of 375 mg/m2 subcutaneously (s.c.) x 4 and plasma exchange.
1.
Two years after having performed anti-CD20 treatment, the patient is with lymphoproliferative syndrome in remission and renal involvement is maintained in the form of residual proteinuria (4 g/day) with conserved renal axis treatment (C.
