Metachronous/concomitant B-cell
neoplasms with distinct morphology are usually considered clonally related. We retrospectively analyzed 4 cases of metachronous/concomitant B-cell
neoplasms with discordant light-chain/heavy-chain restrictions. The primary diagnoses included
chronic lymphocytic leukemia (CLL; n = 2), lymphoplasmacytic
lymphoma (n = 1), and pediatric
follicular lymphoma (FL; n = 1). The respective secondary diagnoses included
diffuse large B-cell lymphoma (DLBCL; n = 2), plasmablastic myeloma, and pediatric FL. The secondary B-cell
neoplasm occurred after the primary diagnosis in 3 cases, with the median interval of 120 months (range, 21-216), whereas the remaining 1 case had the 2
neoplasms (CLL/DLBCL) diagnosed concurrently. Histology suggested aggressive transformation in 3 cases and recurrence in 1 case (FL). Nonetheless, 3 cases showed discordant light-chain restrictions between the 2 B-cell
neoplasms, whereas in the remaining case (lymphoplasmacytic
lymphoma/plasmablastic myeloma), the 2
neoplasms shared κ light-chain restriction but expressed different heavy-chain isotypes (
IgM versus
IgA). The 2 CLL/DLBCL cases had polymerase chain reaction-based IGH/K gene rearrangement study and amplicon sequence analysis performed, which demonstrated distinct clonal amplicons between the 2 B-cell
neoplasms in each case. Concomitant/metachronous B-cell
neoplasms may be clonally unrelated, which can be confirmed by
immunoglobulin isotype analysis and/or genotypic studies. We advocate analysis of clonal identities in large cell transformation or recurrent disease compared with primary indolent B-cell
neoplasm because of a potential difference in prognosis between clonally related and unrelated secondary B-cell
neoplasms.