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

12 th Congress of the European Hematology Association
Our aim was to investigate a possible role of several genetic polymorphisms
in patients with VS after elective CPB. Methods. We performed a
nested case-control study of 50 patients undergoing CPB, 27 men and 23
women, median age 66.5 years (SD 9.6). VS was defined as the conjunction
of a systemic vascular resistance index lower than 1600 dyn seg cm
5 m 2 and a cardiac index greater than 2.5 L/min/m 2 , both occurring within
the first 4 hours after surgery. We recorded data related to hemodynamic
parameters at three different postoperative time points (at Intensive
Care Unit admission, and after 4 and 24 hours from surgery) and the
polymorphism of the following genes: plasminogen activator inhibitor-
1 (PAI-1) and β-tumor necrosis factor (β-TNF). In addition, 22 neutral
markers were genotyped to follow genomic control strategies that would
detect spurious associations due to population substructure. We used
Pearson χ 2 test and binary logistic regression, by means of a SPSS v12.1
informatics software. Results. We observed 17 patients (34%) with vasoplegia
criteria, 11(65%) men and 6 (35%) women, median age 67 years
(range 61-72). Only PAI-1 polyporphism was found to be associated with
vasoplegia, and its distribution in the whole study population was: 4G/G
genotype in 10 patients (20%), 4G/5G in 26 (52%), and 5G/G in 14
(28%). According to PAI-1 polymorphism, vasoplegia criteria were found
in 1(6%) 4G/G carrier, in 6 (35%) 4G/5G carriers and in 10 (59%) 5G/G
carriers. (p=0.012). The post-hoc power for PAI-1 polymorphism and
vasoplegia was 0.85. Once controlled the temperature, clamping time,
body mass index and ACE-inhibitors, 5G/G genotype was independently
associated with vasoplegia (p=0.017); OR: 24.6 (CI95%: 1.8-342). Conclusions.
PAI-1 polymorphism (specifically homozygous 5G/G) was independently
associated with the onset of vasoplegic syndrome. For the best
of our knowledge, do not exist other publications establishing a possible
relationship between fibrinolysis and vasoplegic syndrome. Although
we consider very interesting these findings, further studies are needed to
confirm them.
References
1. Carrel T, Englberger L, Mohacsi P, Neidhart P, Schmidli. Low systemic
vascular resistance after cardiopulmonary bypass: incidence, etiology,
and clinical importance. J Card Surg 2000;15:347-53.
2. Byrne JG, Leacche M, Paul S, Mihaljevic T, Rawn JD, Shernan SK, et al.
Risk factors and outcomes for vasoplegia syndrome following cardiac transplantation.
Eur J Cardiothorac Surg 2004;25:327-32.
1324
IMMUNE THROMBOCYTOPENIA FOLLOWING INFLUENZA VACCINATION IN A PATIENT
WITH CHRONIC LYMPHOCYTIC LEUKAEMIA
M. Mateos, J.M. Arguiñano, M.A. Ardaiz, Y. Burguete, M.C. Montoya,
V. Jarne, A.M. Redondo, M.J. Paloma, M.A. Labaca, I. Ezpeleta,
F.J. Oyarzabal
Hospital Virgen del Camino, PAMPLONA, Spain
Background. Thrombocytopenia is a common complication of chronic
lymphocytic leukaemia, owing to bone marrow infiltration or immune
disturbance. Influenza vaccine is administered to older population as well
as younger people with occupational risk. It is an infrequent cause of
thrombocytopenia. Case report. We report the case of an 83 year old
female diagnosed of chronic lymphocytic leukaemia in stage A/0, requiring
no therapy. Ten days after influenza vaccination she was admitted
because of massive bruising and oral mucosal bleeding. Vaccination had
been with a polyvalent vaccine (Leti laboratories, Tres Cantos, Spain)
containing inactivated fraccionated viruses from H1N1 and H3N2 strains.
Physical examination showed only pethequiae, bruising and hemorrhagic
bullae in oral mucosa. No enlargement of lymph nodes nor spleen or
liver was found. Platelet count was 1×109 /L, Haemoglobin 122 g/L and
leucocytes 21,4×106 /L, with a predominance of lymphocytes due to
chronic lymphocytic leukaemia. In bone marrow examination megakariocyte
hyperplasia was remarkable, as well as a lymphoid infiltrate of
74% of leukemic cells. Therapy with intravenous methylprednisolone
produced a quick response with disappearance of bleeding and platelet
count reaching 140×109 /L in just five days. Once steroid therapy has been
tapered, platelet count remains to date within normal limits. Discussion.
In Spain, influenza vaccination is administered to the whole population
over 65 years. Thus it is a frequent confounding factor in investigation
of thrombocytopenia. In patients with immune disturbances, such as
chronic lymphocytic leukaemia or transplantation, several agents can
trigger autoimmune phenomena. Influenza vaccine is one of these agents
which can induce a short lived thrombocytopenia with an excellent
response to steroids. Conclusion. When facing an acute thrombocytope-
478 | haematologica/the hematology journal | 2007; 92(s1)
nia, past history of drugs and vaccines must be taken into account, even
in the setting of other diseases as chronic lymphocytic leukaemia where
thrombocytopenia is not uncommon. Thus recent influenza vaccination
must be recorded as a cause of immune thrombocytopenia.
1325
COMBINED BAND 3 DEFICIENCY AND α I/74 SPECTRIN VARIANT RESULT IN A CLINI-
CAL PATTERN COMMON TO HEREDITARY SPHEROCYTOSIS AND ELLIPTOCYTOSIS
C. Vercellati, A. Marcello, E. Fermo, P. Bianchi, P. Portararo, A. Zanella
Fondazione IRCCS Ospedale Maggiore, MILANO, Italy
Background. Hereditary spherocytosis (HS) and elliptocytosis (HE) are
highly heterogeneous haemolytic anaemias caused by defects of vertical
interactions between RBC cytoskeleton and integral domain (Spectrin,
Ankyrin, Band 3 and Protein 4.2 deficiency), and by abnormalities
of horizontal interactions among membrane components (Protein 4.1
deficiency, or Spectrin variants impairing the dimer self-association site).
Although a variety of abnormalities have been described in all the RBC
membrane proteins, rare are the cases with combined spherocytic and
helliptocytic defects. Aim. We describe a case of congenital haemolytic
anaemia due to the copresence of HS and HE. Case. The propositus was
a 68 yrs male of Southern Italian origin with a life-long history of
haemolytic anaemia (Hb 8-9 g/dL), jaundice since birth, splenomegaly
and gallstones. At the age of 37 he was diagnosed as HS. In the last two
years, anaemia became more severe requiring occasional transfusions.
Methods. The diagnosis of membrane defect based on clinical history,
physical examination and results of laboratory tests: complete blood
count, blood smear examination, reticulocytes count, bilirubin concentration,
RBC osmotic fragility tests, flow-cytometric eosin-5-maleimide
(EMA) test, and by the exclusion of other causes of haemolysis. Sodium
dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) analysis
of RBC membrane proteins, and functional studies of spectrin were
performed to establish the biochemical defect. The codifying region,
promoter and flanking intronic regions of Band 3 gene (SLC4A1), and
exon 2 of α-spectrin (SPTA1) were amplified and sequenced on an
ABIPRISM 310 capillary sequencer to identify the molecular defect.
Results. At the time of the study Hb was 6.9 g/dL, MCV 84.7 fL, reticulocytes
337×109 /L, unconjugated bilirubin 3.6 mg/dL, LDH 1059 U/L,
haptoglobin