Related Experiment Videos
Factor V inhibitor associated with cold agglutinin disease
D J van Spronsen1, J D Oosting, J J Hoffmann
1Department of Internal Medicine, Catharina Hospital, Eindhoven, The Netherlands.
Annals of Hematology
|March 5, 1998
Summary
A rare case report details an elderly man with cold agglutinin syndrome who developed a cerebral hemorrhage due to a factor V inhibitor, linked to lymphoproliferative disease.
Area of Science:
- Hematology
- Clinical Medicine
- Immunology
Background:
- Cold agglutinin syndrome (CAS) is a rare autoimmune hemolytic anemia.
- Lymphoproliferative disorders can be associated with various hematological complications.
- Coagulation factor inhibitors are uncommon but can cause significant bleeding.
Observation:
- An 81-year-old male with stable CAS presented with progressive cerebral hemorrhage.
- Coagulation tests showed prolonged activated partial thromboplastin time (APTT) and prothrombin time.
- Severely decreased factor V activity was observed, unresponsive to plasma normalization.
Findings:
- The patient was refractory to fresh-frozen plasma, indicating a specific circulating inhibitor against factor V.
- This is the first reported instance of lymphoproliferative disease causing CAS and a factor V inhibitor.
- The findings suggest a link between paraproteinemia, bleeding diathesis, and specific coagulation factor inhibitors.
Implications:
- Clinicians should consider specific coagulation factor inhibitors in patients with paraproteinemia and bleeding.
- This case highlights a novel association between lymphoproliferative disorders, CAS, and acquired factor V deficiency.
- Further research is warranted to understand the mechanisms underlying inhibitor formation in such cases.