12th Congress of the European Hematology ... - Haematologica

Thrombosis II
0814
UNSUSPECTED PULMONARY EMBOLISM: IMPACT ON CANCER PATIENTS' SURVIVAL
P. Rodriguez Otero, R. Lecumberri, R. García Muñoz, E. Ruiz de
Gaona, E. Rocha, J.A. Páramo
University clinic of Navarra, PAMPLONA, Spain
Background. The association between cancer and thrombosis is well
established. The development of a thrombotic episode, either deep
venous thrombosis (DVT) or pulmonary embolism (PE), significantly
relates with worse cancer prognosis and shorter survival. With the use
of multidetector computed tomography (MDCT) for the staging and
follow-up of cancer patients, the diagnosis of incidental PE is increasing,
but its clinical relevance in cancer patients is yet unknown. Aims. To
describe the clinical features and outcomes of adult cancer patients diagnosed
with unsuspected PE using MDCT, comparing completely asymptomatic
patients and patients with non-specific symptoms subsequently
attributed to PE. Methods. Retrospective analysis of consecutive cancer
patients diagnosed with unsuspected PE by MDCT performed for
disease staging or follow-up. Clinical data and D-dimer levels were
assessed at diagnosis. Statistical analysis was performed using Chisquare
tests with SPSS v11.0 software. Results. Between February 2002
and September 2006, 63 cancer patients (mean age 58±12 years, 47.6%
males and 52.4% females) were diagnosed with incidental PE. The most
frequent histologic type of cancer was adenocarcinoma (58.7%), especially
gastro-intestinal. Most patients (66.7%) had metastatic disease at
the time of the PE diagnosis. 58.7% of unsuspected PE were found at
main pulmonary arteries, 27% at lobar arteries, while 14.3% were at segmental
or subsegmental level. Clinical records showed that 58.7% of
patients presented clinical symptoms that could be attributed to PE. During
follow-up (median 17 months), 27 patients (42.9%) died, most of
them due to cancer progression. Mortality was significantly lower in
completely asymptomatic patients than in patients with non-specific
symptoms subsequently attributed to PE (29% vs 57%; p=0.032). In adition,
mortality was related to higher D-dimer levels at diagnosis (607
ng/mL vs 474 ng/mL; p=0.048). No significant association between mortality
and age, sex, cancer stage or subtype or PE location was found.
Conclusions. The absence of clinical symptoms of PE in cancer patients
with unsuspected PE diagnosed by MDCT seems to be associated with
a better prognosis of the neoplastic disease than patients with symptoms
later attributed to PE. pecial attention to signs and symptoms suggestive
of PE in this population is required, in order to achieve an early diagnosis
and treatment. D-Dimer levels could be prospectively evaluated as
prognostic markers after diagnosis of unsuspected PE.
0815
COINHERITANCE OF FV-LEIDEN MUTATION AND FV-HR2
P. Makris, M.P. Makris, S.I. Papamichos, A. Kanidis, P.E. Makris
Aristotle University of Thessaloniki, THESSALONIKI, Greece
The activated protein C resistance (APCr) phenotype is found approximately
in 40% of thrombophilic patients. The factor V gene Leiden
(Arg506Gln) mutation is considered interesting, looking for other factor
V gene mutations associated to thrombophilia: The HR2 haplotype, the
factor V Cambridge (Arg306Thr), the factor V Hong Kong (Arg306Gly)
and the FV Liverpool (Ile359Thr). Because both factor V R506Q and the
HR2 haplotype are very frequent, the effect of their coinheritance on the
risk of venous thromboembolism may present a clinically relevant issue,
and screening for HR2 in carriers of factor V R506Q should be considered.
For this purpose we studied the presence of mutations in a group
of 90 symptomatic patients (who suffered from one or more thromboembolic
events). DNA was extracted from whole blood using the Qiagen
extraction kit and polymorphism was determined by PCR method.
Using the CVD strip assay, we found out that 22 patients were carriers
of the FV Leiden mutation, 7 of the HR2, 10 of the G20210A polymorphism
of prothrombin, 19 of the L34 mutation of XIII, 8 of the b-Fib
455G>A of fibrogen and 12 were homozygote (4G/4G) of PAI-1. Among
these patients, a young individual was found to be double heterozygote,
carrying FV Leiden and FV HR2. This, 21 year-old patient, suffered
an episode of pulmonary embolism. A gene mutation test took place,
because his mother in the age of 47 presented a thrombosis of the left
hand and she was found to be heterozygote for the FV Leiden mutation.
His grandmother passed away in the age of 68 because of ischemic
stroke. Haemostasis control was normal, except from the pathological
value of aPCR (39 sec, normal values >120 sec). The patient was prescript
antivitamin-k for a long-term treatment. Double heterozygosity for factor
V R506Q and HR2 conferred a 3- to 4-fold increase in the relative risk
of venous thromboembolism compared with factor V R506Q alone. No
increase in risk of venous thromboembolism could be demonstrated
when the HR2 haplotype was associated with inherited thrombophilic
defects other than factor V R506Q. The median age at first event was
lower when the 2 defects were associated (46 v 52 years).
0816
RISK FACTORS AND SEQUELAE OF CEREBRAL VEIN THROMBOSIS:
A FIVE YEAR RETROSPECTIVE AUDIT
D. Costello, 1 M. Tharmabala, 2 F. Ni Ainle, 2 M. Spooner, 2 N. Appleby, 2
P. Murphy2 1 2 Beaumont Hospital, DUBLIN; Haematology Dept. Beaumont Hospital,
DUBLIN, Ireland
Background. Cerebral vein thrombosis (CVT) is a rare cause of neurological
dysfunction that has, in recent years been diagnosed more frequently,
in part due to improved non-invasive diagnostic techniques. A
recent meta-analysis that re-evaluated the true natural history of CVT
has identified a lower mortality rate and long-term prognosis than previously
reported. The annual incidence is approximately 3-4 cases per
million 1 however it can be associated with significant morbidity. A prothrombotic
risk factor or direct cause is found in 85% of patients. The
risk of sinus thrombosis is increased in the last trimester of pregnancy
and after delivery 2 and the frequency of peripartum and postpartum
sinus thrombosis is 12 cases per 100,000. 3 A study by Cantu et al. concluded
that CVT during pregnancy and puerperium, while of more acute
onset with a progressive course is often benign 2 Little data exists on the
incidence of recurrent sinus thrombosis in women who have had a first
event. In one series of 68 cases, no recurrent events occurred. 5 A systematic
review by Dentali et al. showed the incidence of recurrence to be
2.8% in a combined cohort of almost 1100 patients. Almost ninety percent
of surviving patients either recover fully or have only a mild functional
or cognitive deficit. 6 Objectives. In the light of this recent metaanalysis,
we evaluated risk factors, morbidity and mortality associated
with CVT in a single Irish centre. Patient and methods. A retrospective
audit was performed of patients diagnosed with CVT in an Irish hospital
during January 2001 to July 2006, evaluating presenting symptoms,
risk factors, survival and neurological sequelae. Results. Eighteen patients
were identified, with a median age of 31 years. The commonest presenting
symptoms were headache, vomiting and visual disturbance. 77%
had at least one risk factor. 39% of events were associated with oral
contraceptive use, pregnancy or postpartum state. 11% were associated
with a local infection. 6% experienced a recurrent CVT. 6% died as
a direct result of the event. 23% developed permanent neurologic deficit.
Conclusions. Our study identified similar risk factors and rates of morbidity
and mortality to those in recent literature, demonstrating that CVT
has a more benign natural history than previously suspected.
References
12 th Congress of the European Hematology Association
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haematologica/the hematology journal | 2007; 92(s1) | 305