Highlights
- •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.
Abstract
Introduction
We describe our experience with managing an unusual case of acquired Factor V deficiency
(aFVd) in a myeloma patient with demonstrated amyloidosis.
Methods
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.
Results
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.
Conclusion
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.
Keywords
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Article info
Publication history
Published online: February 04, 2018
Accepted:
January 26,
2018
Received in revised form:
January 15,
2018
Received:
November 8,
2017
Identification
Copyright
© 2018 Elsevier Ltd. All rights reserved.
