Acquired factor V inhibitor (AFVI) results from the formation of autoantibodies to coagulation factor V (FV), and the clinical phenotype can range from asymptomatic laboratory abnormalities to life-threatening bleeds. We describe a 74-year-old man who developed AFVI along with a massive subcutaneous hematoma. He was initially treated with prednisolone (PSL), but AFVI recurred when the dose was reduced after a short period. We subsequently increased the PSL dose and added cyclophosphamide (CY), which resulted in a complete response. We then gradually tapered PSL and stopped CY, and the patient has since remained free of recurrent AFVI symptoms. We monitored FV activity, antigen concentrations, and inhibitor titers of this patient throughout the clinical course. The ratio of FV activity to antigen concentration was low at diagnosis and gradually increased along with the patient’s improvement. This ratio might be a useful parameter for evaluating the effects of immunosuppressive therapy in patients with AFVI.

1.
Franchini
M
,
Lippi
G
.
Acquired factor V inhibitors: a systematic review
.
J Thromb Thrombolysis
.
2011
May
;
31
(
4
):
449
57
.
[PubMed]
0929-5305
2.
Nogami
K
.
The basic and pathophysiology of the acquired coagulation disorder
.
[Japanese.]
.
Rinsho Ketsueki
.
2013
;
54
:
1907
14
.
[PubMed]
0485-1439
3.
Wang
X
,
Qin
X
,
Yu
Y
,
Wang
R
,
Liu
X
,
Ji
M
, et al.
Acquired factor V deficiency in a patient with a urinary tract infection presenting with haematuria followed by multiple haemorrhages with an extremely low level of factor V inhibitor: a case report and review of the literature
.
Blood Coagul Fibrinolysis
.
2017
Jun
;
28
(
4
):
334
41
.
[PubMed]
0957-5235
4.
Sekiguchi
Y
,
Yoshikawa
H
,
Shimada
A
,
Imai
H
,
Wakabayashi
M
,
Sugimoto
K
, et al.
Acquired factor v inhibitor developing after treatment with dabigatran etexilate methanesulfonate: a case report and review of the literature
.
Indian J Hematol Blood Transfus
.
2014
Sep
;
30
(
S1
Suppl 1
):
275
9
.
[PubMed]
0971-4502
5.
Imashuku
S
,
Hasegawa
T
,
Kubo
K
,
Nakato
M
,
Shima
M
.
Anti-Factor V inhibitor in patients with autoimmune diseases: case report and literature review
.
Int Med Case Rep J
.
2011
Apr
;
4
:
31
4
.
[PubMed]
1179-142X
6.
Bayani
N
,
Rugina
M
,
Haddad-Vergnes
L
,
Lelong
F
.
High-titer acquired factor V inhibitor responsive to corticosteroids and cyclophosphamide in a patient with two malignant tumors
.
Am J Hematol
.
2002
Sep
;
71
(
1
):
33
6
.
[PubMed]
0361-8609
7.
Sakai
M
,
Amano
K
,
Ogawa
Y
,
Takami
A
,
Tokugawa
T
,
Nogami
K
, et al.
Guidelines for the management of acquired hemophilia A: 2017 revision
.
[Japanese.]
.
Nihon Kessen Shiketsu Gakkai shi
.
2017
;
28
(
6
):
715
47
. 0915-7441
8.
W Collins P
. Chalmers E, Hart D, Jennings I, Liesner R, Rangarajan S et al. United Kingdom Haemophilia Centre Doctors' Organization: Diagnosis and management of acquired coagulation inhibitors: a guideline from UKHCDO. Br J Haematol.
2013
; 162: 758-773
9.
Collins
P
,
Baudo
F
,
Huth-Kühne
A
,
Ingerslev
J
,
Kessler
CM
,
Castellano
ME
, et al.
Consensus recommendations for the diagnosis and treatment of acquired hemophilia A
.
BMC Res Notes
.
2010
Jun
;
3
(
1
):
161
8
.
[PubMed]
1756-0500
10.
Emori
Y
,
Sakugawa
M
,
Niiya
K
,
Kiguchi
T
,
Kojima
K
,
Takenaka
K
, et al.
Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis
.
Blood Coagul Fibrinolysis
.
2002
Sep
;
13
(
6
):
555
9
.
[PubMed]
0957-5235
11.
Sosa
IR
,
Ellery
P
,
Mast
A
,
Neff
AT
,
Gailani
D
.
Acquired factor V deficiency in a patient without evidence of a classical inhibitor
.
Haemophilia
.
2014
Jan
;
20
(
1
):
e81
3
.
[PubMed]
1351-8216
12.
Ogawa
Y
,
Yanagisawa
K
,
Uchiumi
H
,
Ishizaki
T
,
Mitsui
T
,
Gouda
F
, et al.
Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan
.
Int J Hematol
.
2017
Jul
;
106
(
1
):
82
9
.
[PubMed]
0925-5710
13.
Ajzner
E
,
Balogh
I
,
Haramura
G
,
Boda
Z
,
Kalmár
K
,
Pfliegler
G
, et al.
Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding
.
J Thromb Haemost
.
2003
May
;
1
(
5
):
943
9
.
[PubMed]
1538-7933
14.
Jeimy
SB
,
Krakow
EF
,
Fuller
N
,
Tasneem
S
,
Hayward
CP
.
An acquired factor V inhibitor associated with defective factor V function, storage and binding to multimerin 1
.
J Thromb Haemost
.
2008
Feb
;
6
(
2
):
395
7
.
[PubMed]
1538-7933
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.