Abstract
BACKGROUND: Acquired factor V deficiency is a rare secondary hemorrhagic disease, which can lead to a severe bleeding disorder. CASE SUMMARY: We report a 47-year-old hemodialysis patient who presented with severe hemorrhagic pleural effusion and hemorrhagic pericardial effusion associated with lymphatic leakage. The laboratory examination revealed decreased factor V activity (2% of population average value). With decreased lymphatic leakage, factor V activity increased (to 46%). Lymph drainage correlated with prothrombin time and active partial thrombin time. The cause of the disease favored an acquired disease. The common causes which trigger factor V inhibitors were excluded. An inhibitor was not detected. It is possible that there was a clotting factor inhibitor leaking with the lymph in the drainage. Inhibitor production may be due to immune dysfunction caused by persistent lymphatic drainage, or that coagulation inhibitors were produced, drained with the lymph, and partly cleared by hemodialysis. CONCLUSION: In this case, we have firstly reported factor V deficiency associated with lymphatic leakage in a hemodialysis patient.