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Blood, 15 March 2008, Vol. 111, No. 6, pp. 3137-3144.

Nonstochastic pairing of immunoglobulin heavy and light chains expressed by

chronic lymphocytic leukemia B cells is predicated on the heavy chain CDR3

F. Widhopf, II1,2, Craig J. Goldberg1,3, Traci L. Toy1,3, Z.

Rassenti1,3, G. Wierda1,4, C. Byrd1,5, J. Keating1,4,

G. Gribben1,6, Kanti R. Rai1,7, and J. Kipps1,3

1 Chronic Lymphocytic Leukemia Research Consortiuim, La Jolla, CA; 2 Division of

Hematology/Oncology, Department of Medicine, and 3 s Cancer Center,

University of California San Diego, La Jolla; 4 Department of Leukemia,

University of Texas M. D. Cancer Center, Houston; 5 Division of

Hematology and Oncology, The Ohio State University, Columbus; 6 Cancer Research

UK Medical Oncology Unit, Barts and The London School of Medicine, London,

United Kingdom; and 7 Division of Hematology/Oncology, Long Island Jewish

Medical Center, New Hyde Park, NY

We analyzed the immunoglobulin (Ig) variable heavy (IGHV) and variable light

chain genes used by leukemia cells of 258 unrelated patients with chronic

lymphocytic leukemia (CLL) found to express unmutated Ig heavy chains (IgH)

encoded by a 51p1 allele of IGHV1-69 among 1846 CLL patients examined. We found

each had at least 98% homology to an identified germline IGKV or IGLV gene.

Within the 258 IgH, we identified heavy chain CDR3 (HCDR3) motifs encoded by

certain unmutated IGHD and IGHJ genes with restricted reading frames. Frequent

and restricted use of particular IGKV and IGLV genes revealed nonstochastic

pairing of disparate Ig light chains (IgL) with IgH that had restricted HCDR3

motifs designated CLL69A, -B, -C, and -D. Eighty-six percent (19/22) of CLL

cases that expressed motif CLL69B encoded by IGHD2-2/IGHJ6 had distinctive IgL

encoded by IGKV1-39. Similarly, 83% (5/6) of samples with motif CLL69D encoded

by IGHD2-2/IGHJ6 expressed IGKV3-11, 100% (25/25) with motif CLL69A encoded by

IGHD3-16/IGHJ3 used IGKV3-20, and 77% (10/13) with motif CLL69C encoded by

IGHD3-3/IGHJ6 expressed IGLV3-9. This study reveals nonstochastic pairing of IgH

with particular IgL that is predicated upon Ig HCDR3 structure, providing

compelling evidence for selection of antibodies expressed in CLL by conventional

antigens.

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