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Chronic Lymphocytic Leukemia with Associated λ-light-chain and IgG λ Paraproteins Simulating a Biclonal Gammopathy by Salvatore Guastafierro, Maria Celentano, Cristina Cuomo, Umberto Falcone

Background: Monoclonal components (MCs) are frequently detected in the sera of patients with B-cell malignancies, by techniques that are getting more and more sensitive. Only few chronic lymphocytic leukemia (CLL) patients with multiple serum paraproteins are reported in the literature.
Methods: In this case report we present a 71-year-old woman with CLL and serum MCs. Immunofixation was performed on agarose film using anti-sera monospecific for the heavy and light chains of human immunoglobulins (anti-γ, -α, -μ, -δ, -ε, -κ, -λ). Serum free light chains (FLCs) were quantified nephelometrically. Immunofluorescence analysis was performed using fluorochrome-conjugated goat antibodies specific for human μ, γ or α immunoglobulin heavy chains and κ or λ light chains.
Results: Immunofixation revealed two different MCs (IgGλ + λ light-chains) in the serum and only one MC (λ light chains) in concentrated urine. Serum λ FLCs were 206 mg/L. The bone marrow aspiration and biopsy revealed a 38 % interstitial and nodular infiltration of mature small lymphocytes expressing IgG λ surface immunoglobulins CD 19, CD20, CD5, and CD23, with negative BCL-1, t(11, 14) and cyclin D1. The plasma cells were less than 1 %. Final diagnosis was CLL (Rai stage I) with IgG λ plus λ serum paraproteins. Three years later, the patient died because of myocardial infarction after a follow-up period with no need for CLL therapy.
Conclusions: Our hypothesis is that the double MC may be the result of an unbalanced immunoglobulin chain synthesis by the leukemic B-cell clone, resulting in IgGλ and excess of λ FLCs.

DOI: Clin. Lab. 2010;56:577-580