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IgG-secreting lymphoplasmacytoid leukaemia: a B-cell disorder with extensively mutated VH genes undergoing Ig isotype-switching frequently associated with trisomy 12

IgG-secreting lymphoplasmacytoid leukaemia: a B-cell disorder with extensively mutated VH genes undergoing Ig isotype-switching frequently associated with trisomy 12
IgG-secreting lymphoplasmacytoid leukaemia: a B-cell disorder with extensively mutated VH genes undergoing Ig isotype-switching frequently associated with trisomy 12
We investigated 16 patients with elevated serum monoclonal IgG and a leukaemic B-cell lymphocytic disorder different from multiple myeloma. Their clinical history was that of a non-aggressive disease with dominant splenomegaly and long survival. Whereas abnormal blood and bone marrow cells were predominantly small lymphocytes with a few lymphoplasmacytoid cells, histopathological features included a lymphoplasmacytic infiltrate in eight cases. Most frequently, abnormal blood cells displayed a CD19+CD5-CD23+/- immunophenotype different from that of chronic lymphocytic leukaemia, except in two cases with a CD19+CD5+CD23+ phenotype. Interestingly, a coexistent serum monoclonal IgM and/or surface IgMG+ with identical light chain was identified in 10 patients, whereas in the remaining six patients only IgG expression was determined. VH gene analysis was performed in eight patients to investigate the clonal origins of tumour cells. All cases utilized the VH3 family, with evidence of extensive somatic mutations and intraclonal homogeneity in all cases. VH gene analysis indicated a clonal relationship between cells expressing IgM and IgG, with one case being biclonal. Cytogenetic evaluation showed a high incidence of trisomy 12 (60%) and 13q14 deletion (40%). In conclusion, we have described an unusual subset of low-grade lymphoma with high-serum IgG and frequent lymphoplasmacytoid features in which tumour cells derive from post-follicular memory B cells undergoing isotype switching with some cases arrested at both the IgM and IgG stage and others as IgG-positive cells only.
0007-1048
71-80
Garand, R.
81283b21-6266-4a65-85e0-1c5c30c40c39
Sahota, S.S.
66c1457f-cba2-4c49-9c8c-fcee0748b6b8
Avet-Loiseau, H.
149ad87f-9358-4109-8843-4b2bc9087402
Talmant, P.
6e18a073-fbeb-47b5-94ef-11a9dc8de976
Robillard, N.
13ae0ff1-90b3-47ae-979f-f8d7b6268737
Moreau, A.
e7ef260e-7129-4ec2-9e47-9b915cab09fe
Gaillard, F.
359e2235-b574-4a8f-9d5e-4760a98d2b9e
Stevenson, F.K.
ba803747-c0ac-409f-a9c2-b61fde009f8c
Bataille, R.
daa64563-0713-45c0-8e56-a0e0dba70b89
Garand, R.
81283b21-6266-4a65-85e0-1c5c30c40c39
Sahota, S.S.
66c1457f-cba2-4c49-9c8c-fcee0748b6b8
Avet-Loiseau, H.
149ad87f-9358-4109-8843-4b2bc9087402
Talmant, P.
6e18a073-fbeb-47b5-94ef-11a9dc8de976
Robillard, N.
13ae0ff1-90b3-47ae-979f-f8d7b6268737
Moreau, A.
e7ef260e-7129-4ec2-9e47-9b915cab09fe
Gaillard, F.
359e2235-b574-4a8f-9d5e-4760a98d2b9e
Stevenson, F.K.
ba803747-c0ac-409f-a9c2-b61fde009f8c
Bataille, R.
daa64563-0713-45c0-8e56-a0e0dba70b89

Garand, R., Sahota, S.S., Avet-Loiseau, H., Talmant, P., Robillard, N., Moreau, A., Gaillard, F., Stevenson, F.K. and Bataille, R. (2000) IgG-secreting lymphoplasmacytoid leukaemia: a B-cell disorder with extensively mutated VH genes undergoing Ig isotype-switching frequently associated with trisomy 12. British Journal of Haematology, 109 (1), 71-80. (doi:10.1046/j.1365-2141.2000.01971.x).

Record type: Article

Abstract

We investigated 16 patients with elevated serum monoclonal IgG and a leukaemic B-cell lymphocytic disorder different from multiple myeloma. Their clinical history was that of a non-aggressive disease with dominant splenomegaly and long survival. Whereas abnormal blood and bone marrow cells were predominantly small lymphocytes with a few lymphoplasmacytoid cells, histopathological features included a lymphoplasmacytic infiltrate in eight cases. Most frequently, abnormal blood cells displayed a CD19+CD5-CD23+/- immunophenotype different from that of chronic lymphocytic leukaemia, except in two cases with a CD19+CD5+CD23+ phenotype. Interestingly, a coexistent serum monoclonal IgM and/or surface IgMG+ with identical light chain was identified in 10 patients, whereas in the remaining six patients only IgG expression was determined. VH gene analysis was performed in eight patients to investigate the clonal origins of tumour cells. All cases utilized the VH3 family, with evidence of extensive somatic mutations and intraclonal homogeneity in all cases. VH gene analysis indicated a clonal relationship between cells expressing IgM and IgG, with one case being biclonal. Cytogenetic evaluation showed a high incidence of trisomy 12 (60%) and 13q14 deletion (40%). In conclusion, we have described an unusual subset of low-grade lymphoma with high-serum IgG and frequent lymphoplasmacytoid features in which tumour cells derive from post-follicular memory B cells undergoing isotype switching with some cases arrested at both the IgM and IgG stage and others as IgG-positive cells only.

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Published date: April 2000

Identifiers

Local EPrints ID: 157803
URI: https://eprints.soton.ac.uk/id/eprint/157803
ISSN: 0007-1048
PURE UUID: 2abae33b-cf8f-4ce0-8eb6-6b25303160e1
ORCID for F.K. Stevenson: ORCID iD orcid.org/0000-0002-0933-5021

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Date deposited: 06 Jul 2010 13:01
Last modified: 06 Jun 2018 13:01

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