Acquired factor V deficiency in a patient with myeloma and amyloidosis

• Published in: Thrombosis research Vol. 164; pp. 1 - 3
• Main Authors: Quek, J.K.S., Wong, W.H., Tan, C.W., Tha, Mya Hae, Nagarajan, C., Lee, L.H., Ng, H.J.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Ltd 01.04.2018
• Subjects: Amyloidosis; Factor V deficiency; Inhibitor titer; Plasma cell dyscrasia; Amyloidosis; Factor V deficiency; Plasma cell dyscrasia; Inhibitor titer
• ISSN: 0049-3848; 1879-2472
• DOI: 10.1016/j.thromres.2018.01.045

We describe our experience with managing an unusual case of acquired Factor V deficiency (aFVd) in a myeloma patient with demonstrated amyloidosis. Following diagnosis, records of previous investigations were sought. Specific clotting factors and inhibitors were tested. The clinical progress and treatment response measured by serial factor V levels and coagulation parameters was then prospectively tracked. A 57 year-old woman presented with spontaneous right knee haemarthrosis in association with bilateral symmetrical polyneuropathy and proteinuria. Coagulation screen showed prolongation of both PT (18.6 s, normal range [9.9–11.4 s]) and aPTT (41.4 s, normal range [25.7–32.9 s]), which were both fully correctable following a mixing study. Liver function, fibrinogen, clotting factor II/VIII/X assays and disseminated intravascular coagulopathy screen was normal. FV level was reduced (19%, normal range [70–170%]). Inhibitor titer was undetectable. Congenital FVd was excluded as her previous coagulation screen was normal. Bone marrow investigation performed for suspected underlying plasma cell dyscrasia showed 60% neoplastic plasma cells. Congo red staining was positive for amyloid within vascular walls of the marrow trephine. She was diagnosed with light chain myeloma and aFVd. She received Bortezomib/Cyclophosphamide/Dexamethasone (VCD) chemotherapy. After one cycle of VCD, serum kappa free light chain (SFLC) was reduced from 6951 mg/L to 3354 mg/L with serial measurements of FV levels showing increment to 76% and normalization of PT/aPTT. Plasma cell dyscrasia with amyloidosis should be sought as a cause for aFVD, in particular one where bleeding manifestation is profound even with the absence of demonstrable inhibitors. •Acquired factor V deficiency (aFVd) is a rare bleeding disorder commonly caused by autoantibodies against factor V.•Isolated acquired factor V deficiency associated with plasma cell dyscrasia is rare.•In the absence of a demonstrable inhibitor, the mechanism of aFVD acquired could be either from adsorption of factor V by amyloid deposits or mediated by light chains that were significantly elevated.•Plasma cell dyscrasia should be sought as a cause for aFVD, in particular one where bleeding manifestation is profound.