A Child with severe Congenital Factor VII Deficiency,
And Factor VII alloantibody inhibitor
Eshghi P1, Abolghasemi H² ,Madani F ³, Habibpanah B4
Correspondence to: Dr Peyman Eshghi ,Email:Peyman64@yahoo.com
Dr Hassan Abolghasemi:H.abolghasemi@sbmu.ac.ir
Dr Farhad Madani:Dr.fmadani@sbmu.ac.ir
1. Professor of Pediatric Hematology & Oncology, Pediatric Congenital Hematologic Disorder Center, Shahid Beheshti
Medical University, Tehran, Iran
2. Professor of Pediatric Hematology & Oncology, Pediatric Congenital Hematologic Disorder Center, Shahid Beheshti
Medical University, Tehran, Iran
3. Pediatric Hematology & Oncology Fellowship, Pediatric Congenital Hematologic Disorder Center , Shahid Beheshti
Medical University, Tehran, Iran
4. B.s of Nursing, Comprehensive Care Center for Children with Hemophilia, Mofid Children Hospital, Shahid Beheshti
Medical University, Tehran, Iran
Introduction:
Deficiency of factor VII comprises a highly heterogeneous disease group, but a few cases
lacking factor VII function entirely or subtotally may not present with a history of bleeding.
When factor VΠ: C levels are very low the bleeding episodes can occur frequently. The most
frequent bleedings are menorrhagia and metrorrhagia in females and hemarthrosis in both
sexes. .Inhibitor against Factor seven this rare disease is even more infrequent,so that there
were not more than 5 reported case in registered patients globaly,that makes it more intense
and need proposals to debate on management of these patients. In this article we are going to
report the occurrence of Allo antibody against Factor VII in a child with severe congenital
Factor VII deficiency leading to clinical problems.
Cases Report:
A 4 years old girl who was a known case of Factor VII deficiency that was referred to Mofid
Comprehensive Care Center for Children with Hemophilia(MCCCH) because of severe headache and
somnolence ,she had SAH in her Brain CT scan which did not need surgical intervention according to
neurosurgical consult, and should be just under clinical observation.
In previous history she was the first child of healthy related parents .her mother’s first and second
pregnancy were miscarried .The child was managed at 3th month of life because of gross hematuria
as UTI and VUR. Since then she had 3 times of left knee bleeding until the diagnosis were confirmed
at 3 years old for her with coagulation laboratory data.(Table-1) Then after she has been treated Ondemand with high doses of rFactor VIIa -about 100 mcg/kg/dose .Since 5 months ago she
experienced more episodes of hemorrhagic events conduce to hospitalization need to high
dose rFVIIa .Mean while she had 2 times of severe flushing skin reactions to rFVIIa recently in
another hospital .unfortunately she has received both available brands of rFVIIa during her
management(Aryoseven® and Novoseven-RT®) and there was not any more documentation about it.
In current admission in our hospital we found that not only she did not respond well to standard dose
(30-40 mcg /kg/dose) but also the PT did not even normalized with high dose of medication.
She evaluated for Factor seven inhibitor that was 80 BU. Because of some reactions such as
itching ,skin rashes following the rFVIIa and not a good response to high dose treatment we
tried to control her symptoms with FEBA .Her Joint and CNS bleeding Symptoms were
managed with 50-75 IU/kg Q8-12 hr and finally 80-100 U/kg Once daily for about 2 weeks.
Now she is on a 75/kg -3 times/weekly regimen prophylactically.
Lab Data
Factor VII level
PT
PTT
PT Control
Plt
Factor VII Inhibitor (BU)
Factor VII Inhibitor (BU)
during therapy
<1%
35
23
13.6
442000
80
Emission
Table -1
Discussion:
In this paper we report A case with inhibitors against factor VII on the long and shortterm use of factor VII Recombinant .that describes the inhibitor as a terrible bother during
management of Bleeding attacks In factor VII deficiency.
Few independent published reports are available about Factor VII deficiency inhibitors and
how to manage it. Unfortunately efficacy of high dose Factor seven is not clear. When no
alternative treatment was available or risk of thromboembolism could be seen. Few other
inhibitors have been reported in previously published studies from STERS. Or Inadequate
Inhibitors might be suspected in patients display abnormally Hemostatic effects of treatment
with high dose factor seven recombinant.
Keywords:
FVII deficiency, Factor VII inhibitor, FVII Recombinant, FEIBA.