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

12 th Congress of the European Hematology Association
Saturation=16.7±2.8, ALT=76. The follow up was conducted for
47.7±13.9 days; at its end: RBC=4.7±0.11; Hb=14.4±0.4; PLT=231±17;
PT=97.8±2.4; Ferritin=39.5±5.6 and Transferrin Saturation=25.9±3.7,
ALT=73. gammaGT was respectively 62, 52 and 55 U/L (p=0.011
between time 0 and 1; p=0.043 between time 0 and 2). Total bilirubin significantly
decreased between time 0 and 1 (0,81 and 0,56 mg/dL, p=0.002)
and gone up at time 2 (0.56, p=0.055) but not significantly. Plasmatic
albumin, prothrombinic activity and ALP showed not significant variations.
From a clinical point of view, patients reported to tolerate the treatment,
to feel a generic sensation of well-being and to feel itself less tired
anlighter; main problems were: the difficulty in finding the vein, the pain
linked to vein puncture, a sensation of malaise and an headache during
and immediately after the phlebotomy. Conclusions. On the basis of our
experience, with a weekly frequency of phlebotomy it is possible to complete
all therapeutic cycles without causing an anemia in patients an
avoiding complications and adverse reactions. Final follow-up showed
that iron depletion is maintained for a long time thanks to stimulation of
bone marrow hemopoiesis, due to iatrogenic anemia. Moreover we have
registered a very positive action on hepatic inflammation parameters,
probably linked to removal of the iron excess that acts as further pathogenic
factor upgrading the cytopathic effects of HCV. Although clinical
response and laboratory results must be always monitored, we can affirm
that blood letting, in removing a liver cytopathic factor as iron overload,
can be favorable for patients affected with hemosiderosis secondary to
hepatitis; moreover, using opportune and appropriate sagacities, iron
depletion does not represent a limiting factor for specific antiviral treatments
determining an anemization of patient.
1330
REMISSION OF REFRACTORY CHRONIC IMMUNE CYTOPENIAS WITH RITUXIMAB
A. Müller, 1 A. Soyano, 2 A.E. Soyano, 3 H. Goldstanj, 4 M. Di Stefano, 1
M. Melendez, 1 N. Noriega, 1 A. Urbaez, 5 A. Hong6 1 Inst. of Hematology & Oncology (MS-UCV), CARACAS; 2 Venezuelan Inst.
for Scient. Research, CARACAS; 3 Luis Razetti Medical School (UCV), CARA-
CAS; 4 Hospital de Clinicas Caracas, CARACAS; 5 Luis Razetti Hospital,
BARCELONA; 6 Hospital de Ninos, CARACAS, Venezuela
Background. Chronic inmune cytopenias refractory to conventional
treatment represent a therapeutic challenge. Recent studies have shown
that rituximab might be useful in the treatment of these patients due to
its B-cell depleting effect. Aims. The objective of this study was to evaluate
the effect of rituximab in immune cytopenias. Patients and Methods.
Twenty-eight (28) patients with chronic immune cytopenia refractory
to other treatments were treated with rituximab: 15 patients with chronic
immune thrombocytopenic purpura (ITP), 1 patient with thrombotic
thrombocytopenic purpura, 9 with recurrent autoimmune hemolytic
anemia (AIHA) and 3 with Evans syndrome. Patients (5 children and
ten adults) with ITP for up to 21 years (2 of the patients also with diagnostic
of LED), 6 to 78 years-old, with platelet counts 150,000/µL was achieved and partial (PR) if 50,000 to
150,000/µL. Nine adult patients (21-78 years-old) with refractory
autoimmune hemolytic anemia, eight of them with direct Coombs test
(IgG) positive and one patient with direct Coombs test (IgM) positive
and three patients (14 to 43 years-old) with Evans syndrome were also
treated with Rituximab. In these cases CR was characterized as complete
if normal Hb and Hto for their sex and age was achieved and PR if their
Hb increased at least two grams. Study of CD20 + B cells was done with
monoclonal antibodies by flow cytometry. RESULTS: Thirteen patients
(11 adults and 2 children) with refractory ITP responded to rituximab de
novo (86.7%). Four of them had been splenectomysed. Twelve (12)
patients had been in CR for 6 months to 2.7 years (8 pts in CR for more
than a year) and 1 patient in partial remission for 1 year. Nine (9) out of
12 patients (75%) who entered in CR relapsed after de novo treatment
with rituximab. Seven of these patients were retreated with rituximab
and all of them have responded for up to 1.7 years. The two children
with ITP did not respond. Eight patients with AIHA have been in CR
for 1 to 2 years. One patient 21 year old with AIHA had a CR only for
one month. Only one patient with AIHA has relapsed; he was retreated
responding again to rituximab. Three patients with Evans syndrome
have been in CR for 2 months to 2 years. Therapy was well tolerated.
The CD20 + count decreased to less than 1% after rituximab. Conclusions.
In our series, most patients with refractory chronic immune
cytopenias responded to de novo rituximab treatment or to retreatment,
480 | haematologica/the hematology journal | 2007; 92(s1)
even if they have been splenectomized. Rituximab may be considered
a good treatment option, allowing the withdrawal of steroids when the
patients enter in CR and thus reducing steroid side effects for a reasonable
period of time. The AIHA patients had less number of relapses with
rituximab than the ITP patients. The fall in CD20 count talks in favor of
depletion of CD20 positive B cell as a mechanism of action of rituximab.
1331
METASTATIC EPITHELOID HAEMANGIOENDOTHELIOMA IN A 22 YEAR OLD PATIENT
C. Sippel, 1 H.-J. Holzhausen, 2 K. Jordan, 2 W. Voigt, 2 H.-J. Schmoll, 2
H.-H. Wolf2 1 University Hospital Halle, HALLE; 2 University Hospital, HALLE, Germany
Background. Eptheloid hemangioendothelioma (EH) is a rare neoplasia
of vascular origin involving soft tissue and visceral organs. Lesions
occur in almost all ages except early childhood without preference in
gender. Usually EH develops as solitary tumour of the soft tissue either
of superficial or visceral origin involving skull, axial skeleton or lower
extremities and low progression. Because of the rare occurrence of the
disease and its granulomatous histology, approximately 2/3 of EH
patients may be misdiagnosed initially. Patient’s characteristics. We report
a 22 year old male patient who presented with multiple granulomatous
lesions of lung and liver. Initially the patient complained pain of the
right arm and progredient dyspnoe for almost 2 years. Radiologic examination
of the chest showed bilateral lymphadenopathia. During the last
20 months the patient had underwent several biopsies of lung and liver
in regional hospitals, and sarcoidosis had been diagnosed. The patient
had been treated with prednison for 12 months without any improvement
of cough or dyspnoe or regression of the lymphadenopathy. Finally,
liver biopsy revealed diagnosis of EH. In March 2007, we started
chemotherapy according to the sarcoma protocol with ifosfamide, adriamycine,
actinomycine and vincristine, Conclusion. Even if EH is a rare
tumour, it should be considered in patients with granulomatous lesions
of soft tissue. It is not proven whether new monoclonal antibodies might
improve prognosis in metastatic disease.
1332
IS THERE A PREDICTIVE ROLE FOR GA-67 SCINTIGRAPHY IN PATIENTS WITH
LYMPHOMA UNDERGOING AUTOLOGOUS STEM CELL TRANSPLANTATION?
P. Kiratli, 1 P. Kiratli, 1 B. Erbas, 1 E. Ozdemir, 1 Y. Koc2 1 Hacettepe University, ANKARA; 2 Yeditepe University, ISTANBUL, Turkey
Aims. The aim of this study is to evaluate Gallium-67 scintigraphy
(GS) and CT for predicting clinical outcome in lymphoma patients who
undergo autologous stem-cell transplantation (ASCT). Methods. Forty
patients undergoing ASCT, had GS before and at 100 days post-transplantation
(D-100). Patients were followed for 6-61 months and 15 had
repeat GS at 200 days post-transplantation (D-200). All patients underwent
CT imaging. Results. Out of 40 patients, 15 were diagnosed with
HD and 25 with NHL. Fifteen had pathologic D-100 GS, where 7 were
correlated with CT. Out of 8 patients with normal CT, 6 had control
studies. Two patients returned to normal, 1 showed persistence and 3
were accepted in relapse due to progressive lesions on GS with new
appeared lesions on CT. Four patients with pathologic findings on D-100
CT but normal GS, were in remission on follow-up. Of the patients
85.7% with negative and 28% with positive D-100 GS were disease-free
(median follow up: 30 months). The PPV, NPV and accuracy for GS were
64%, 88% and 77.5%, respectively, and 37.5%, 75% and 75% for CT,
suggesting importance of D-100 GS in the prediction of outcome in lymphoma
patients who undergo ASCT. Conclusions. The results of our findings
suggest that GS is superior compared to CT for the prediction of
progression, so it can be used in centers where PET imaging is not available.
1333
SERUM UREA, A SURROGATE MARKER FOR IMPENDING VASOOCCLUSIVE CRISIS
IN SICKLE CELL DISEASE
A. Pathare, 1 S. Alkindi, 2 N. Nusrut, 3 A. Pathare, 3 R. Krishnamoorthy4 1 SQUH, MUSCAT, Oman; 2 College of Medicine & Health Sciences, SQU,
MUSCAT, Oman; 3 Department of Haematology, MUSCAT, Oman; 4 INSERM
U763, Hopital Robert Debre, PARIS, France
Background. Vasoocclusive crisis (VOC) is the outcome of several interactions
between sickle red blood cells, the endothelium and leucocytes