A Case of Haemorrhages caused by Multiple Myeloma Induced Factor Deficiencies: A Bleeding Enigma

Abstract

A 74-year-old male patient presented with anaemia, thrombocytopenia, and extensive haemorrhages over both thighs and back. A coagulation work-up revealed markedly elevated Prothrombin Time (PT) and activated Partial Thromboplastin Time (aPTT). Reduced functional values of fibrinogen as determined by clot-based assay (126.4 mg/dL), as well as factor V (17.5%) and factor X (27.8%), were noted. These abnormalities were corrected by mixing studies with normal plasma. Neoplastic plasma cells were seen in the bone marrow biopsy, while abdominal fat biopsies showed no amyloid deposition. Serum electrophoresis and immunofixation were positive for monoclonal gammopathy. The patient was diagnosed with multiple myeloma complicated by multiple acquired factor deficiencies. The patient showed a complete response within six months of treatment, which included bortezomib and cyclophosphamide. Multiple myeloma is a monoclonal proliferation of plasma cells that results in increased production of free immunoglobulin light chains, resulting in increased paraproteins. These paraproteins bind with and inhibit various clotting factors, resulting in their inactivation and depletion. Factor X deficiency in these cases is usually attributed to concomitant amyloidosis, whose fibrils bind to Factor X. However, these deficiencies can occur in the absence of amyloidosis as well. While various reports of this entity have highlighted isolated factor deficiencies including factors V, VII, IX, and X, multiple factor deficiency is a rare occurrence, with no other cases being reported from southern India.

Keywords

• bleeding disorders
• factor deficiency
• haematology