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Chronic Lymphocytic Leukemias

IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL)

Leukemia volume 20, pages 1231–1237 (2006)Cite this article

Abstract

B-prolymphocytic leukemia (B-PLL) is a rare disease with poor prognosis. To further characterize the biological features of this disease, we analyzed immunoglobulin heavy chain (IgVH) mutations, ZAP-70 and CD38 in 19 cases with de novo B-PLL. Immunoglobulin heavy chain genes analysis showed an unmutated pattern (>98% homology to germ line) in 9/17 cases (53%), with 100% homology in eight. In the remaining, it ranged from 90 to 97.4%, with three cases slightly mutated (98–95%) and five heavily mutated (<95%). All B-PLL utilized members of VH3 (11/17) and VH4 (6/17) families, with V3-23, V4-59 and V4-34 gene accounting for more than half of them, regardless of mutational status. ZAP-70, assessed by flow cytometry, ranged from 1 to 91% cells, being ⩾20% in 57% of cases. CD38 ranged from 1 to 99% (median 21%). There was no correlation between IgVH status and ZAP-70 or CD38 expression, but male gender and del(17p) were more common in the unmutated group. Neither IgVH mutations, CD38 expression nor del(17p) influenced patients’ outcome. Unexpectedly, ZAP-70+ B-PLL patients survived longer (40 months) than ZAP-70− B-PLL (8 months). B-PLL appears biologically heterogeneous regarding IgVH mutations, ZAP-70 and CD38 expression, showing a pattern distinct from that of other lymphoproliferative disorders.

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Author notes

  1. I Del Giudice

    Present address: Dr I Del Giudice, Dipartimento di Biotecnologie Cellulari ed Ematologia, Sezione di Ematologia, Università ‘La Sapienza’, Via Benevento 6, 00161, Roma, Italy

Authors and Affiliations

  1. Section of Haemato-Oncology – Institute of Cancer Research, Sutton, UK

    I Del Giudice, E Matutes, N Osuji, N Parry-Jones, A Morilla, V Brito-Babapulle & D Catovsky

  2. Department of Haematology, Royal Bournemouth Hospital, Bournemouth, UK

    Z Davis & D Oscier

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Correspondence to I Del Giudice.

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Del Giudice, I., Davis, Z., Matutes, E. et al. IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL). Leukemia 20, 1231–1237 (2006). https://doi.org/10.1038/sj.leu.2404238

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