Abstract book 6th RMS 16.indd

subjects reporting a clinical and family
history of bleeding. Abnormal results
of screening tests are followed by the
specific coagulation assays, in order to
make the specific diagnosis of a RBD.
Molecular diagnosis is based on the search
of mutation in the genes encoding the
corresponding factors.
Treatment of RBDs is also a difficult
task, since information on the clinical
management of RBDs is often scarce.
The patients’ personal and family history
of bleeding are important guides for
management. The main treatments in
RBDs are represented by non-transfusional
adjuvant therapies and replacement
therapy of the deficient coagulation factor.
However, evidence-based guidelines for
the diagnosis and management of this
patient population are still lacking.
With these limitations in mind we
established an International network
of treatment centers to report clinical,
laboratory (phenotypic and genetic) and
therapeutic information. The first report
from 495 european patients were analyzed.
There was a strong association between
coagulation factor activity level and clinical
bleeding severity for fibrinogen, FX,
FXIII, and FV+VIII deficiencies. A weaker
association was present for FV and FVII
deficiencies, whereas no association was
present for FXI deficiency. In addition
levels not associated with spontaneous
major bleeding were highest for FXIII
deficiency, followed by FV+VIII, FVII,
FX, and FV deficiencies. For fibrinogen
deficiency, a factor activity level of 20 mg/
dl is needed to ensure absence of major
spontaneous bleeding. Thus, these data
prove a heterogeneous relation between
coagulation factor activity level and clinical
bleeding severity in RBDs. Results from
this study call for further work directed
to allocate suitable preventive and
management strategies for this patients
population.
70
Menorrhagia and Bleeding Disorders:
Management Options
Rezan Abdul- Kadir MD (UK)
blood loss (MBL) can only be measured
precisely by alkaline haematin method
to extract its haemoglobin content. This
is a sophisticated laboratory test and not
practical for clinical use. Pictorial blood
assessment chart (PBAC) is a simple
clinical tool that can be used to assess
the blood loss and monitor response to
treatments. Heavy menstrual bleeding can
be a symptom of an underlying bleeding
disorders and haemostatic evaluation
should be considered in women with HMB.
However, full haemostatic screen is neither
necessary nor feasible for all women
presenting with HMB. The use of PBAC
score as well as bleeding score helps identify
women who are more likely to benefit
from screening for bleeding disorders.
Iron deficiency anaemia is commonly
associated with HMB. Early detection and
treatment of iron deficiency is important
aspect of the management. Treatment
options for HMB include haemostatic,
hormonal and surgical options. The choice
is dependent on the woman’s age, her
reproductive wishes, the side effect profile
and availability of the treatment options.
Close collaboration between Gynaecology
and haematology teams is essential for
optimal care for women with bleeding
disorders presenting with HMB.
71
Home Treatment In Haemophilia
Assad Haffar MD (Canada)
Early treatment with replacement therapy
is an important key factor in stopping the
bleeding, minimizing complication and
returning of the affected joint or area to
normal as early as possible. In order to
be able to treat early, it advisable to give
each hemophilia few vials of Clotting
Factor Concentrates (CFCs) to keep them
at his residence in order to start treatment
as soon as a bleed starts. This is what is
known as Home Treatment or Home
Therapy which is essential in prophylaxis
and has a lot of benefits in addition to the
concept of early treatment.
Menorrhagia (heavy menstrual bleeding)
is a common gynaecological problem and
has a major impact on women’s general
health and quality of life. Actual menstrual
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