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

12 th Congress of the European Hematology Association
conditional dependency rules, the EORTC EBPGs for erythropoietic proteins.
1207
DEFIBROTIDE IN THE PREVENTION AND TREATMENT OF VENO OCCLUSIVE DISEASE
IN AUTOLOGOUS AND ALLOGENEIC STEM CELL TRANSPLANTATION IN CHILDREN.
A. Qureshi, 1 L. Marshall, 2 D. Lancaster3 1 2 St. Mary's Hospital, LONDON, United Kingdom; Royal Marsden Hospital,
SUTTON, United Kingdom
Background. Veno-occlusive disease (VOD) is a common (10-50%) and
serious complication of stem cell transplantation. Aims. To investigate the
effectiveness of defibrotide as prophylaxis and treatment against VOD
in children which has been shown to effective in adults. Methods. Defibrotide
prophylaxis (20 mg/kg/day) was given to 47 successive patients
who underwent transplantation between April 2004 and December
2005. Incidence of VOD was compared with 56 historical controls transplanted
between November 2001 and April 2004. High risk patients in
the control group (busulphan conditioning and deranged liver function)
received ursodeoxycholic acid, tinzaparin and glutamine as VOD prophylaxis.
The groups were matched for sex, age, type of transplant (unrelated,
related allogeneic or autologous transplant) and risk. Results. The
mean maximum bilirubin was the same in both groups (32 µmol/L). In
the defibrotide group, 2 patients (4%) developed VOD (Seattle criteria).
2 further patients were also treated for presumed VOD but this was not
confirmed on liver biopsy. Defibrotide dose was increased in all 4
patients to 40-60 mg/kg/day. All patients’ symptoms resolved within 14
days and are currently alive 30- 330 days post transplant. No serious side
effects were reported. Of the control group 4 patients (7%) had VOD.
2 of these patients had reversed hepatic vein flow. 3 of these patients
received Busulphan conditioning and 2 died in ITU 30 days post transplant,
partly due to VOD. VOD was associated with busulphan conditioning
( p= 0.001) and not with age, sex, type of transplant, Graft vs
Host Disease, deranged liver function prior to transplant or type of antifungal
prophylaxis. Although our study demonstrates a small overall
incidence of VOD this was reduced in the defibrotide group where there
was also no VOD related mortality. Our study suggests that defibrotide
can be effectively used as prophylaxis and treatment in high risk patients
but further larger multi centre studies should be carried out.
1208
VALUE OF THE MORPHOLOGIC CHARACTERISTICS OF THE BONE MARROW IN THE DIAG-
NOSISI OF ESSENTIAL THROMBOCYTHEMIA: STUDY OF 22 CASES.
O. Xavier, 1 Ll. Pons, 2 M. Rodríguez-Luaces, 3 T. Alvaro, 2 Ll. Font3 1 Hospital de Tortosa Verge de la Cinta, TORTOSA; 2 Anatomía Patológica,
TORTOSA; 3 Hematología, TORTOSA, Spain
Background. The criteria diagnoses of the WHO for essential throboocythemia
(ET) confer value to the histopathology and facilitate more
precise diagnoses in the differentiation of ET of other chronic myeloproliferative
disorders, in special initial stages of myelofibrosis (MF) with a
significant unfavorable prognosis. Aims. Histopathologic revision of cases
initially diagnosed ET and correlation with the clinical and analytical
evolution of them. METHODS Hystopathologic revision of 22 cases of
patients initially diagnosed of ET throughout 14 years was done. Morphologic
criteria are used according to the WHO and the described ones
according to Thiele Et al. (Histol Histopathol. 2005; 20: 633-644). We valued:
cellular density in relation to the age, number of megakaryocytes,
presencet of clusters dense, clusters lax, number of megakaryocytes of
great, intermediate and small size, nuclear lobulation, defects of maturation
(nucleus of bulbous and naked aspect). Also it is made the evaluation
of cellular density and reticulynic fibrosis according to the European
consensus (Thiele et t al. Haematologica 2005; 90:1128 - 32) and
gradation of the MF is made in four degrees. The cellular percentage has
been obtained from digital images by means of the software of analysis
of image Image-Pro Plus ® 5,0 (Average Cybernetics, U.S.A.). Results.
The hystopathologic study of the 22 samples of bone marrow reviewed
shows that, 4 of them - cases 10.11.13 and 18 - (18.18%) present a degree
of increased cellularity according to the age of the patient and to corresponding
by the criterion the initial diagnosis ET. In reference to the
myelofibrosis degree, 2 - cases 18 and 20 - of the 22 reviewed bone
biopsies (9.1%), present a degree of reticulynic fibrosis superior to hoped
for the initial diagnosis of ET. During the pursuit of the patients thrombotic
complications take place in 3 cases (1 cerbrovascular disease, 1
pulmonary embolism (PE) and 1 venous thrombosis/PE). Conclusions.
Applying recent consensus of standardized criteria for the hystophato-
442 | haematologica/the hematology journal | 2007; 92(s1)
logic study of the biopsies of bony marrow, 5 analyzed samples of a total
of 22, (22.72%) corresponding ones to biopsies initially diagnosed of
ET, they could be redefined like pertaining to precocious stages of
myelofibrosis. Even though which in only one of these five cases (case
13) presence of thrombotic and/or hemorragic complications is observed
and a worse control of the numbers of platelets (in relation to the line
number required of treatment) this redefinition diagnoses can be of utility
facing the evolution foretells end of the process.
1209
THE EFFECT OF RADIOLOGICAL IMAGING STUDIES ON THE RISK OF SECONDARY
MALIGNANCY DEVELOPMENT IN PATIENTS WITH HODGKINS LYMPHOMA
C. Beyan, K. Kaptan, A. Ifran, R. Öcal, C. Ulutin, B. Öztürk
Gulhane Military Medical Academy, ANKARA, Turkey
Background. Recently, reports suggesting that diagnostic radiological
imaging studies could play a role on the risk of secondary malignancies
have been published. Nowadays, a guideline about the use radiological
techniques in patients with multiple myeloma have been reported by
UK Myeloma Forum Guidelines Working Group and Nordic Myeloma
Study Group which emphasized unnecessary risk due to X-ray exposure
originating from frequent imaging investigations. Aims. The aim of our
study is to calculate the average amount of accumulated radiation by
means of radiological imaging studies performed intensively in diagnosis
and follow-up of patients with Hodgkin’s lymphoma and to evaluate
whether this amount of accumulation account for a real risk for secondary
malignancies. Methods. This study consists of 15 male patients,
whose mean age was 23,67±4,24 years. All radiological imaging studies
performed in Hodgkin’s lymphoma patients were noted in detail and
average radiation dose accumulation was calculated according to data of
National Radiological Protection Board (NRPB) and Biological Effects of
Ionizing Radiation (BEIR) VII report. Results. Median radiation dose
which patients subjected during median 14,5 months of disease duration
was 85,19 miliSievert (mSv) and 161,08 mSv according to data of NRPB
and BEIR VII report, respectively. The cumulative radiation dose because
of radiological imaging studies is 8,5-16 times greater than that of the
described dose having 1 in 1.000 chance of cancer development according
to BEIR VII report. Approximately, this amount is equivalent to the
dose of natural background radiation received during 35-70 years. Conclusions.
Our study demonstrated that radiation dose accumulation
because of radiological imaging studies used in diagnosis, staging and follow-up
of patients with Hodgkin’s lymphoma was high enough to cause
development of secondary malignancies. Finally, it is obvious that the
radiological imaging study policies used in follow-up of these patients
should be overviewed.
1210
ENDOGLIN AS A NEW BIO INDICATOR OF HAEMORHEOPHERESIS/IMMUNOAPHERESIS
PROCEDURE EFFICIENCY
M. Blaha, 1 M. Cermanova, 1 V. Blaha, 1 M. Blazek, 1 P. Solich, 2 D. Satinsky,
2 L. Smolej, 1 J. Maly, 1 S. Filip1 1 Faculty Hospital, Medical Faculty, HRADEC KRALOVE, Czech Republic;
2 Pharmaceutical Faculty, Charles Univ., HRADEC KRALOVE, Czech Republic
Background. An ideal tool for immediate evaluation of extracorporeal
elimination therapy would be a serum marker, levels of which would
correlate with disease activity and reflect an improvement after elimination
therapy. Still, there are no suitable markers for extracorporeal
elimination in familial hypercholesterolemia (FH) which would reliably
determine the therapy intensity immediately after the procedure and
would be able to show activity of atherosclerosis. Aims. In a previous
study, we found that the levels of P-selectin, MCP-1, hsCRP and CD40L
were reduced after LDL-apheresis. Although the post-elimination
decreases in all four markers reached statistical significance, we speculated
that endoglin (sCD105) levels could represent an even more effective
tool for evaluation of treatment efficacy. Patients and Methods. Altogether
40 examinations of endoglin level in 11 patients with severe FH
and long-term treatment (4.5±2.8 years) by extracorporeal elimination
(LDL-apheresis and haemorheopheresis) were done. Informed consent
was obtained. We measured sCD105 levels immediately before and
after two consecutive elimination procedures. Results. Baseline serum
sCD105 levels were significantly higher in the patients than in the control
group. The decrease in sCD105 after both LDL-elimination series
was also statistically significant. Endoglin level normalized after procedures
in all of 40 except one measurements. Conclusions. We conclude
that endoglin can serve as a useful marker for evaluation of the treatment