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

gle dose of 54mg/m 2 rituximab further stresses the need for further studies
to define the optimal dose and schedule for rituximab therapy in
cITP.
Reference
1. Taube T, et al. Effect of a single dose of rituximab in chronic immune
thrombocytopenic purpura in childhood. Haemotologica 2005; 90:281-3.
1321
HEMOGLOBINOPATHIES AND CONGENITAL HEMOLYTIC ANEMIAS. A COMPARATIVE
STUDY OF AUTOCHTONOUS VS IMMIGRANT POPULATIONS IN SOUTHERN SPAIN. A TEN
YEAR FOLLOW-UP
M.A. Molina, 1 M.J. Giménez, 2 R. Pérez, 3 J.A. García, 3 M.T. Gallego, 3
A.M. Jiménez, 3 M.T. Cabezas, 3 M.I. Cabeza, 3 A.B. Lozano, 3 J. Salas, 3
C. Avivar3 1 Empresa Pública Hospital de Poniente, EL EJIDO ALMERÍA; 2 Transfusion
Center, ALMERÍA; 3 Hospital de Poniente, EL EJIDO ALMERÍA, Spain
Background. An estimated 30% of the world’s population suffers from
anemia. Structural hemoglobinopathies and thalassemias are the monogenic
disorders with the highest prevalence. Migrational social changes
in our country are resulting in a remarkable increase in the occurrence
of these abnormalities, considering both mild (heterozygous) or severe
(homozygous or compound heterozygous) forms. The immigrant population
in Almería (Andalusia, southern Spain) makes up approximately
17.4%, 110,637 out of 635,850 inhabitants. Aims. To describe the cases
of congenital hemolytic anemias occurring in the immigrant population
in our area, mostly coming from The Maghreb and Sub-Saharan
Africa, compared to those detected in the autochtonous Mediterranean
population. Material and methods. Anemia work-up protocols over a ten
years period (from January 1997 to January 2007) have been reviewed.
Since October 2002 we have used the Hi-AUTO A1c 8160 (Menarini-R)
high-pressure liquid cromatography (HPLC) system, which allows the
detection of abnormal variants of hemoglobin, with their final characterisation
made by acidified medium electrophoresis. Results. Out of the
3,149 anemia protocols reviewed, 484 corresponded to immigrant
patients (15.4%). We detected 81 cases of hemoglobinopathy (16.7%),
11 cases of GP6DH deficiency (2.4%) and 10 cases of hereditary spherocytosis
(2%). Among hemoglobinopathies, we found: 20 β thalassemia
minor; 18 hemoglobin (Hb) AS; 13 Hb AC; 6 homozygous HbS (HbSS);
6 heterozygous α thalassemia; 5 β thalassemia intermedia; 5 double heterozygous
HbSC; 5 HbCC; 1 delta β thalassemia; 1 HbSS + α thalassemia
and 1 HbSS + β thalassemia. Since we started using the HPLC abnormal
hemoglobin band detection system: 60 HbAS; 15 HbAC; 4 β thalassemia
minor; 2 persistence of fetal hemoglobin (HPFH) and 1 HbCC have been
added. Considering the autochtonous population, 2,665 anemia protocols
were carried out, which detected 131 hemoglobinopathies (4.9%),
59 hereditary spherocytosis (2.2%) and 7 GP6DH deficiency (0.3%).
Among the hemoglobinopathies were: 68 β thalassemia minor; 23 β thalassemia
intermedia; 23 α thalassemia; 14 delta β thalassemia; 1 HbAC;
1 HPFH and 1 HbAS or HbAD that we were unable to characterise.
Using the HPLC system, we added: 6 Hb AD; 3 Hb AS; 3 Hb AC; 2 delta
β thalassemia; 2 Hb AS or AD;1 β thalassemia intermedia; 1 β thalassemia
minor; 1 PHHF and 1 Hb C or E. Conclusions. 1) Of the anemia
investigation protocols carried out, 15.4% were on the immigrant population,
which is similar to the percentage of the estimated immigrant
population in Almería overall. 2) In immigrants, we have found hemoglobinopathies,
even without anemia, that are unusual in local patients,
and shouldn’t be underdiagnosed. 3) An abnormal hemoglobin band
detection system, such as HPLC, is of great usefulness in the diagnosis
of anemia. 4) Data shown here is general in nature. It would be of great
interest to classify these patients according to their specific origin. 5)
Hereditary spherocytosis, the most prevalent hemolytic anemia among
caucasians, should not be ignored when investigating anemia in immigrants.
12 th Congress of the European Hematology Association
1322
THE USE OF COLLAGEN COATED BLOOD SAMPLING BOTTLES TO DETECT FUNCTIONAL
AND NON FUNCTIONAL PLATELETS IN NORMAL SUBJECTS AND PATIENTS SEEN IN ITU,
HAEMATOLOGY CLINICS AND UNDERGOING RENAL DIALYSIS
J.G. Smith, 1 S. Burford, 1 S. Pambakian, 1 L. Sheridan-Smith, 1
V. Chomyn, 2 D. Skinner, 3 J. Campbell3 1 Frimley Park Hospital, CAMBERLEY, United Kingdom; 2 Horiba ABX,,
CHICKSANDS, United Kingdom; 3 Plateletworks, Helena Laboratories, BEAU-
MONT, TEXAS, USA
Platelet function is an important factor to prevent bleeding irrespective
of circulating number. Classically, platelet function has been assessed
by Simplate bleeding time measurement, platelet aggregometry and,
more recently, PFA100 technology. Despite this, patients often have surgery
cancelled due to the fact that they are taking Aspirin or similar
agents eg. Clopidogrel. In the ITU setting, clinicians often face the situation
where patients are bleeding but the humoral coagulation and
platelet count are not apparent issues. This is particularly the case where
the patient is on extra-corporeal circulation. The concept of exhausted
platelets is real but cumbersome to prove. Therefore, the availabilty of
a techique which can be performed at the bedside which is reliable and
reproducible in enumerating the patient’s functionalplatelet count would
be potentially invaluable. In the last three years Helena Laboratories,
Beaumont, Texas, US have produced blood sampling bottles which are
coated with 10 ug collagen (equine tendon) and 3.2mg sodium citrate
(Plateletworks). Our group is the first in Europe to use these bottles in
association with a point of care testing system (POCT) using the Horiba
ABX Pentra five part differential analyser (Chicksands, Bedfordshire,
England). POCT full blood counts were performed on samples of blood
taken from normal subjects, renal dialysis patients, cardiac catheterisation
patients, ITU patients and haematology patients with myelodysplasia
and myeloproliferative disorders. A small cohort of patients were on
regular low dose aspirin and some of the normal subjects volunteered to
take aspirin prior to testing. One normal subject was on antihistamines.
Blood samples were taken synchronously into K2EDTA and collagen
bottles and both were handled identically in terms of mixing and analysis
on the ABX Pentra. All staff undertaking the study had been trained
by Plateletworks in sample handling. The K2EDTA platelet count represents
the total platelet count whereas the measurable platelet count in
the collagen coated bottle represents the non functional compartment of
the individual’s platelet count ( the functional platelets having aggregated
onto the inside wall of the collagen tube). Data on 54 normal subjects
showed that the interquartile range of functional platelets was between
89.9-95.9%. Median value was 93.8%. This implies that even in healthy
subjects a small proportion of platelets are non functional. Cardiac
catheterisation patients (n=10) all of whom were on Aspirin and Clopidogrel
had 76.5% functional platelets which is statistically significant
when compared to control subjects (p=0.0009). Surprisingly, one subject
who had taken 600mg of Aspirin 24 hours earlier had 70% functional
platelets using this assay system. Finally renal patients who were already
thrombocytopenic had significantly lower functional counts than expected
such that platelet transfusion might be considered. The data presented
here have been generated using collagen coated tubes only. This, at
best, is a screening tool where the most appropriate assessment of
Aspirin effect may be achieved using Arachidonic Acid. Plateletworks is
looking at producing such tubes shortly. Nonetheless, the philosophy of
measuring the functional platelet count is still valid and may provide
haematologists with a valuable tool in deciding treatment in the future.
1323
RELATIONSHIP BETWEEN PAI-1 POLYMORPHISM AND DEVELOPMENT OF VASOPLEGIC
SYNDROME ASSOCIATED WITH CARDIOPULMONARY BYPASS AFTER CARDIAC SURGERY
J.M. Raya, 1 J.M. Raya, 2 J.M. Rodríguez-Martín, 1 J.J. Jimenez, 1
J.L. Iribarren, 1 M.J. Rodríguez-Salazar, 1 M. Brouard, 1 T. Martín, 1
P. Reina, 1 P. Machado, 1 R. Perez, 1 P. Garrido, 1 M.L. Mora, 1 R. Martínez1 1 Hospital Universitario de Canarias, LA LAGUNA - TENERIFE; 2 Spanish
Hematocytology Group, LA LAGUNA - TENERIFE, Spain
Introduction. Vasoplegic syndrome (VS) is a recognized complication following
cardiac surgery using cardiopulmonary bypass (CPB) and is associated
with increased morbidity and mortality. Intensity of VS can vary
from mild to severe and it occurs with an incidence ranging between 5%
and 15%. Although its etiology is not completely understood, risk factors
as temperature, duration of CPB and preoperative treatment with
angiotensin-converting enzyme (ACE)-inhibitors have been reported. 1,2
haematologica/the hematology journal | 2007; 92(s1) | 477