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

esulting alterations of the endothelium with release of vasoactive substances
and cytokines. Recognizing clinical subsets in sickle cell disease
(SCD) with biomarkers, is a useful strategy in deciphering secondary
factors involved in modulating the SCD phenotype. There is no single
clinical or laboratory test that can prognosticate SCD patients, making
it is difficult to stratify patients in relation to disease severity and outcome
of several complications. Aims. The aim of this study was to assess
the significance of alterations in the serum urea concentrations in steady
state and VOC’s in patients with SCD and use it as a surrogate marker
to monitor VOC. Methods. SCD patients (n=58; 48 SS homozygotes;10
S‚+ Thal double heterozygotes) admitted from the A/E in VOC were
enrolled prospectively after informed consent. All patients were also
studied later in steady state. The period between the painful crises, during
which the patient feels well, is called steady state. i.e no acute illness
or VOC or infection in the previous 3 months. Complete blood counts
were obtained with an electronic cell counter (Abbott CELL-DYN 4000,
Abbott Diagnistics, Abbott Park, IN). A fresh hemolysate was prepared
from each sample and subjected to cation-exchange high-performance
liquid chromatography (Bio-Rad VARIANT, Bio-Rad Laboratories, Hercules,
CA) to reconfirm the hemoglobin phenotype. C-reactive protein
(CRP) was estimated by rate nephelometry. Several biochemical parameters
of renal and liver function were measured by Beckman Synchrom
CX7 analyzer. Serum urea was estimated in all of these patients in the
steady state, at the time of presentation of acute painful crisis (before
receiving any fluid hydration), at 48 hrs of the VOC episode, and before
discharge. Results. The patients comprised of 25 male [29 episodes] and
33 female [44 episodes]. Four males and five females had two episodes,
while 3 females had 3 admission episodes. Patients age ranged from 14
year to 43 years (mean ± SD, 22.15±6.24). It was observed that mean
serum urea (±SD) in the steady state, at 2.8 (±0.07) dropped significantly
at the time of presentation of VOC to 1.8 (±1.1; p< 0.0005; Student’s
t-test) (Table 1). Summary/Conclusions. Our study strongly indicates the
value of monitoring serum urea concentrations during the VOC
episodes. We therefore hypothesize that the urea cycle is shifted towards
a reduced urea production, which also leads to a decreased production
of nitrous oxide. This compound is a well known vasodilator and its
reduced availability is recognized to play an important role in the pathogenesis
of SCD crisis. It is hypothesized that serum urea levels will correlate
with the L-arginine nitric oxide pathway and can be a useful biomarker
to monitor the progress of VOC episodes. Furthermore, it can be
also useful in prognostication of SCD complications.
Table 1. Mean S. urea in Steady state, at Admission, 48 hrs Inpatient and
at Discharge.
1334
LOW DOSE THALIDOMIDE AS MAINTENANCE IN MULTIPLE MYELOMA
M. El Sorady, A. Elghandour
Faculty of Medicine, ALEXANDRIA, Egypt
Background. Autologous hematopoietic stem cell transplantation
(ASCT) following chemotherapy improves survival and decrease relapse
rate in patients with multiple myeloma. However, patients not eligible
for ASCT relapse earlier demanding search for new treatment modalities.
Aim. was to evaluate the effect of low dose thalidomide as a maintenance
treatment in patient with first complete remission not candidate
for autologous bone marrow transplantation, and its effect on disease
free survival. Patients. the study was carried out on 102 patients randomized
in two groups, group I (44 patients) and group II (48 patients). Group
I received 50 mg thalidomide daily, and group II did not receive any
maintenance treatment. Results. the follow up period was 40 months,
median age for group I was 62 years and 64 years for group II. Males to
12 th Congress of the European Hematology Association
females ratio was 3:1 for both groups. Regarding types of myeloma; IgG
kappa myloma represent 70% in group I and 75% in group II, IgG lambda
20% and 19% in group I and II respectively. Out of 44 patients in
group I, only 12 patients relapsed during follow up period, 9 of them
died, while 40 patients relapsed from group II, 26 of them died with a
significant difference between both groups (p