- •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.
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.
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Published online: February 04, 2018
Accepted: January 26, 2018
Received in revised form: January 15, 2018
Received: November 8, 2017
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