Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association
Abstract Background Lupus anticoagulant (LA) is an in vitro phenomenon with prolongation of a phospholipid-dependent coagulation test which is not due to an inhibitor specific to a coagulation factor. Occasionally, addition of normal pooled plasma to patient plasma with lupus anticoagulant potentiat...
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2024-12-01
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| Series: | Thrombosis Journal |
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| Online Access: | https://doi.org/10.1186/s12959-024-00680-x |
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| author | Ting Hon Stanford Li Pui Lun Yip Hui Yiu Chan Ho Kin Stephen Chan Wai Shan Wong |
| author_facet | Ting Hon Stanford Li Pui Lun Yip Hui Yiu Chan Ho Kin Stephen Chan Wai Shan Wong |
| author_sort | Ting Hon Stanford Li |
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| description | Abstract Background Lupus anticoagulant (LA) is an in vitro phenomenon with prolongation of a phospholipid-dependent coagulation test which is not due to an inhibitor specific to a coagulation factor. Occasionally, addition of normal pooled plasma to patient plasma with lupus anticoagulant potentiates the inhibitory effect of lupus anticoagulant in the mixture, resulting in a paradoxical prolongation instead of shortening of clotting time. The phenomenon has been termed the “lupus cofactor effect”. Lupus anticoagulant are known to be associated with lymphoma and immunoglobulin M (IgM) paraproteinaemia. Cases of lymphoplasmacytic lymphoma with concomitant IgM paraproteinaemia and lupus anticoagulant demonstrating lupus cofactor phenomenon on activated partial thromboplastin time (APTT) assay has been reported previously. However, to our best knowledge, there were no reported cases of low grade B-cell lymphoma with positive LA results and lupus cofactor effect demonstrated on dilute Russell’s viper venom time (DRVVT) and/or silica clotting time (SCT) assays in the literature. Case presentation We report two cases of low grade B-cell lymphoma associated with monoclonal IgM paraprotein, high levels of anti-cardiolipin IgM antibody and presence of lupus anticoagulant with lupus cofactor phenomenon on DRVVT and/or SCT assay. Conclusions Our cases demonstrate a possible novel association between low grade B-cell lymphoma, IgM paraproteinaemia, high levels of anti-cardiolipin IgM antibody and the presence of lupus cofactor effect on DRVVT and SCT assays. The DRVVT assay in the first patient and SCT assay in second patient were falsely negative in the neat sample or less diluted sample, and the lupus anticoagulant activities were only revealed on dilution of samples with normal pooled plasma. This highlights the potential importance of dilution of samples with normal pooled plasma while evaluating the LA status of low grade B-cell lymphoma patients with a markedly prolonged APTT and/or prolonged PT by DRVVT and SCT assays, especially if there is concomitant IgM paraproteinaemia and a high level of anti-cardiolipin IgM antibody. |
| format | Article |
| id | doaj-art-de060efb93ee4f23bc560ee1c019f2be |
| institution | Kabale University |
| issn | 1477-9560 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | BMC |
| record_format | Article |
| series | Thrombosis Journal |
| spelling | doaj-art-de060efb93ee4f23bc560ee1c019f2be2024-12-08T12:41:31ZengBMCThrombosis Journal1477-95602024-12-012211610.1186/s12959-024-00680-xLupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel associationTing Hon Stanford Li0Pui Lun Yip1Hui Yiu Chan2Ho Kin Stephen Chan3Wai Shan Wong4Department of Pathology, Queen Elizabeth HospitalDepartment of Medicine, Queen Elizabeth HospitalDepartment of Pathology, Queen Elizabeth HospitalDepartment of Pathology, Queen Elizabeth HospitalDepartment of Pathology, Queen Elizabeth HospitalAbstract Background Lupus anticoagulant (LA) is an in vitro phenomenon with prolongation of a phospholipid-dependent coagulation test which is not due to an inhibitor specific to a coagulation factor. Occasionally, addition of normal pooled plasma to patient plasma with lupus anticoagulant potentiates the inhibitory effect of lupus anticoagulant in the mixture, resulting in a paradoxical prolongation instead of shortening of clotting time. The phenomenon has been termed the “lupus cofactor effect”. Lupus anticoagulant are known to be associated with lymphoma and immunoglobulin M (IgM) paraproteinaemia. Cases of lymphoplasmacytic lymphoma with concomitant IgM paraproteinaemia and lupus anticoagulant demonstrating lupus cofactor phenomenon on activated partial thromboplastin time (APTT) assay has been reported previously. However, to our best knowledge, there were no reported cases of low grade B-cell lymphoma with positive LA results and lupus cofactor effect demonstrated on dilute Russell’s viper venom time (DRVVT) and/or silica clotting time (SCT) assays in the literature. Case presentation We report two cases of low grade B-cell lymphoma associated with monoclonal IgM paraprotein, high levels of anti-cardiolipin IgM antibody and presence of lupus anticoagulant with lupus cofactor phenomenon on DRVVT and/or SCT assay. Conclusions Our cases demonstrate a possible novel association between low grade B-cell lymphoma, IgM paraproteinaemia, high levels of anti-cardiolipin IgM antibody and the presence of lupus cofactor effect on DRVVT and SCT assays. The DRVVT assay in the first patient and SCT assay in second patient were falsely negative in the neat sample or less diluted sample, and the lupus anticoagulant activities were only revealed on dilution of samples with normal pooled plasma. This highlights the potential importance of dilution of samples with normal pooled plasma while evaluating the LA status of low grade B-cell lymphoma patients with a markedly prolonged APTT and/or prolonged PT by DRVVT and SCT assays, especially if there is concomitant IgM paraproteinaemia and a high level of anti-cardiolipin IgM antibody.https://doi.org/10.1186/s12959-024-00680-xLupus anticoagulantLupus cofactor effectLow grade B-cell lymphomaIgM paraproteinaemia |
| spellingShingle | Ting Hon Stanford Li Pui Lun Yip Hui Yiu Chan Ho Kin Stephen Chan Wai Shan Wong Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association Thrombosis Journal Lupus anticoagulant Lupus cofactor effect Low grade B-cell lymphoma IgM paraproteinaemia |
| title | Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association |
| title_full | Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association |
| title_fullStr | Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association |
| title_full_unstemmed | Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association |
| title_short | Lupus anticoagulant associated with low grade B-cell lymphoma and IgM paraproteinaemia with lupus cofactor phenomenon on DRVVT and SCT assays - a possible novel association |
| title_sort | lupus anticoagulant associated with low grade b cell lymphoma and igm paraproteinaemia with lupus cofactor phenomenon on drvvt and sct assays a possible novel association |
| topic | Lupus anticoagulant Lupus cofactor effect Low grade B-cell lymphoma IgM paraproteinaemia |
| url | https://doi.org/10.1186/s12959-024-00680-x |
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