Dramatically prolonged coagulation screening tests in a patient with positive lupus anticoagulant and monoclonal immunoglobulin M without bleeding manifestations

Background: Concomitant presence of lupus anticoagulant (LA) and monoclonal immunoglobulin in the same patient is uncommon and the influence of this finding on coagulation results is still unknown. Case report: We present a patient with a diagnosis of systemic lupus erythematosus (SLE) and secondary antiphospholipid syndrome (APS) with permanently positive LA who presented with accidental finding of newly discovered monoclonal IgM in a high concentration and dramatically prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT), without bleeding manifestations. Conclusion: Concomitant presence of extremely prolonged PT and aPTT with unusual coagulation reaction kinetics, consistent LA ratio over the follow-up period and normalization of coagulation screening results with decreasing monoclonal IgM concentration elicited suspicion that PT and aPTT prolongation could be attributed to M-protein with antiphospholipid specificity. Low LA-sensitive aPTT reagent Actin FS demonstrated exceptional sensitivity, whereas human placental thromboplastin in contrast to recombinant reagents showed significantly lower sensitivity to monoclonal IgM with antiphospholipid specificity. Changes in the activity of SLE observed during the follow-up period were inversely related to monoclonal IgM concentration, while the presence of secondary APS was consistent. Described analytical interference on PT and aPTT without bleeding manifestation should point towards suspicion of previously unidentified monoclonal IgM with antiphospholipid sensitivity.

Automated summary

The concomitant presence of lupus anticoagulant (LA) and monoclonal immunoglobulin in the same patient is uncommon. In this case, the patient showed dramatically prolonged prothrombin time and activated partial thromboplastin time without bleeding manifestations. The normalization of coagulation screening results with decreasing monoclonal IgM concentration suggests a potential relationship between monoclonal IgM and the prolonged coagulation results.