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

12 th Congress of the European Hematology Association
compared to controls (Figure1). The levels of CD41 [80.62% vs.
86.31%, p=0.290] and CD42a [77.98% vs. 94.15%, p=0.99] demonstrate
no significant differences in the two groups. Granulophysin
(CD63) expression was negatively correlate with hemathocrit level
(Ro=-0.447, p=0.037). Interactions between renal function determinants
(BUN, uric acid, creatinine), coagulation indices (APTT, INR),
clinical determinants (fever, cutaneous haemorrhage), treatment
options (chemotherapy, antibiotic therapy) and platelets functionality
were analysed without significant results. Due to relative reduced
number of cases no correlation could be established with AML subtype
or evolving stage of the disease. Conclusions. The AML patients present
changes in adhesion receptors, i.e. decreasing of CD42b (von Willebrand
factor) and activation markers, CD63 (granulophysin) and
CD62P (P-selectin), suggesting a functional defect or a denatured intracellular
signalling. The exposed data indicates that flow cytometry can
effectively identify multiple platelet impairments regarding adhesion,
aggregation, and secretion associated with in AML pathogenesis, and
express the need for furthermore extensive investigations in order to
integrate these platelet behaviour features in AML clinical context.
Figure 1. Expression of platelet surface markers.
502 | haematologica/the hematology journal | 2007; 92(s1)
1399
IMMUNE THROMBOCYTOPENIC PURPURA: A 10 YEAR EXPERIENCE FROM A SINGLE
CENTER
V. Tsiora, E. Hatzimichael, A. Vassou, L. Benetatos, V. Zina, K. Skandalis,
E. Oikonomou, E. Kapsali, K.L. Bourantas
University Hospital of Ioannina, IOANNINA, Greece
Background. Immune thrombocytopenic purpura (ITP) is a common
acquired autoimmune disorder. The natural course of ITP is unknown
and no standard therapeutic approach exists for the one third of patients
who develop refractory disease. Aim of this study was to examine the
clinical features, the laboratory findings and the effects of different treatment
modalities in patients with ITP and to draw any useful conclusions.
Methods. 59 patients (37 female, 22 male, age range 33-94 years, mean
61.7 years) who were diagnosed at our center between 1996 and 2006
were evaluated retrospectively. The diagnosis of ITP was based on history,
physical examination and decreased platelet count with an otherwise
unremarkable peripheral smear. All other causes of thrombocytopenia
were excluded. Treatment was initiated when the platelet count
was less than 30×109 /L Relapse was defined as a blood platelet count less
than 30×109 /L and chronic refractory ITP was defined as the failure of
any modality to keep the platelet count above 20×109 /L for an appreciable
time without unacceptable toxicity. Results. 33 patients (56%) of
patients were asymptomatic at diagnosis and thrombocytopenia was
found on a routine visit to a physician. Thirteen patients (22%) presented
with easy bruising and/or petechiae, 12 (20%) patients with minor
bleeding, such as gingival bleeding, epistaxis, macroscopic hematuria
and one patient with severe intracerebral hemorrhage. Thirty-one
patients (17%) had a positive autoimmune profile with main findings
being positive antinuclear antibodies, antiplatelet antibodies and low
C¬¬3. None of the patients developed an autoimmune disorder. With
regard to therapy 3 patients received no treatment; as first line treatment
13 patients received steroids only and 43 patients received combination
of prednisolone (1mg/kg) and IVIg (400 mg/kg) for 4-5 days. In 24
patients (40%) no relapse was noted. Out of the 45 patients who
relapsed 12 patients (20%) experienced one relapse, 11 patients (18.6%)
2 relapses and 12 patients (20%) had more than 2 relapses. In patients
with symptomatic relapse or severe thrombocytopenia steroids and IVIg
was reintroduced and 12 of them achieved remission. Third line treatment
consisted of danazol (10 patients), splenectomy (6 patients), iv
anti-CD20 (8 patients), azathioprine (2 patients) and vincristine (4
patients). In 4 patients who relapsed post splenectomy iv anti-CD20
was given at a dose of 375 mg/m2 weekly for 4-6 consecutive weeks. All
12 patients who received anti-CD-20 responded to treatment achieving
a platelet count >50×109 /L, however 3/12 patients relapsed 7. 9 and 24
months post infusion. Adverse effects of therapy were allergic reaction
to IVIg (2 patients), iatrogenic Cushing and myopathy secondary to
steroids (4 patients) and pneumonococcal pneumonia in a splenectomized
patient. Results. ITP is an autoimmune disorder with variable
clinical presentation and course. Patients with chronic refractory ITP are
difficult to manage. AntiCD-20 therapy appears as a promising
immunomodulatory agent for the management of these patients.
1400
BRAIN MRI FINDINGS IN LEBANESE SICKLE CELL DISEASE PATIENTS
A. Inati Khoriaty, 1 H. Tarabay, 2 H. Moallem, 3 Y. Rachkidi, 2
R. Wehbe, 2 O. Jradi, 4 S. Koussa, 5 A. Taher6 1 2 Rafik Hariri University Hospital, BEIRUT; Nini Hospital, TRIPOLI, Lebanon;
3 4 Lebanese American University, BYBLOS; Labib Medical Center, SIDON;
5 6 Chronic Care Center, BAABDA, Lebanon; American University of Beirut,
BEIRUT, Lebanon
Background. Sickle cell disease(SCD) is one of the most prevalent
hemoglobinopathies in Lebanon. Acute stroke defined as an acute neurological
syndrome secondary to arterial occlusion or hemorrhage with
resultant neurological symptoms and signs lasting for more than 24
hours is the most catastrophic brain injury seen in this disorder and has
a prevalence of around 10% by 50 years of age. Silent cerebral infarcts
(SCI), defined as an abnormal magnetic resonance image (MRI) of the
brain and no history of neurologic deficit, are seen in 20-44% of children
with sickle cell anemia (SCA) and represents a major risk factor for overt
stroke. Identifying asymptomatic patients at high risk of developing
sickle-related brain injury helps physicians implement preventive and
effective therapies. Aims. 1. Determine prevalence of brain imaging
abnormalities in a group of Lebanese patients with SCD and 2. Identi-