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

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mild. Fas and soluble Fas ligand levels were found to be higher on day<br />

0, when compared to day 15 levels and this finding suggests that Fas-Fas<br />

ligand apototic pathway is active during <strong>the</strong> acute phase <strong>of</strong> <strong>the</strong> VZV<br />

infection and remits after recovery <strong>of</strong> illness. NK cell count and activity,<br />

perforin expression, Fas and soluble Fas ligand levels will be analyzed in<br />

future studies in severely complicated VZV infected patients.<br />

Financially supported by, Hacettepe University, Bilimsel Arastirmalar Kurumu<br />

(project number: 05 D11 101 007)<br />

1258<br />

CIRCUMCISION IN PATIENTS WITH HEMOPHILIA: TEN YEARS EXPERIENCE IN ADANA,<br />

TURKEY<br />

I. Sasmaz, B. Antmen, G. Leblebisatan, R. Tuncer, Y. Kilinc<br />

Cukurova University Medical Faculty, ADANA, Turkey<br />

Fiftyty-one boys with hemophilia (age range, 6 months to 18 years),<br />

37 (72,5%) with hemophilia A and 14 (27,5%) with hemophilia B were<br />

circumcised in Cukurova University, Faculty <strong>of</strong> Medicine, Department<br />

<strong>of</strong> Pediatric <strong>Hematology</strong>, between 1997 and 2007. Two <strong>of</strong> children with<br />

hemophilia have inhibitors. 12 (23,5%) cases were mild, 24 (47,0%) cases<br />

were moderate and 15 (29,5%) cases were severe hemophilia. After<br />

starting systemic prophylaxis including factor substitution and DDAVP<br />

(desmopressin acetate) in for reducing factor doses, 5 patients underwent<br />

circumcision under local anes<strong>the</strong>sia and 46 patients underwent circumcision<br />

under general anes<strong>the</strong>sia. All patients were given tranexamic<br />

acid. Duration <strong>of</strong> <strong>the</strong> hospitalization period was 2 to 7 days according<br />

to whe<strong>the</strong>r or not complication. Transient minimal bleeding was<br />

observed in 23 (45%) patients and easily responded to factor administration.<br />

No life treatening hemorrahges were observed. Minimal local<br />

edema and hyperemia along <strong>the</strong> excision line was observed in all<br />

patients. The objective <strong>of</strong> this retrospective study was to reported <strong>the</strong><br />

experience <strong>of</strong> circumcision in patients with hemophilia in <strong>the</strong> Cukurova<br />

University, Faculty <strong>of</strong> Medicine, Department <strong>of</strong> Pediatric <strong>Hematology</strong>.<br />

1259<br />

INTENSIFIED IMMUNOCHEMOTHERAPY WITH HIGH DOSE CONSOLIDATION<br />

AND AUTOLOGOUS STEM CELL RESCUE IN MANTLE CELL LYMPHOMA<br />

M. Binder, R. Ziebermayr, O. Krieger, H. Kasparu, M. Girschik<strong>of</strong>sky,<br />

D. Lutz<br />

Elisabethinen Hospital Linz, LINZ, Austria<br />

Background. Mantel cell lymphoma (MCL) is an aggressive B- cell lymphoma<br />

which is characterised by early dissemination and unfavourable<br />

clinical course. The implementation <strong>of</strong> autologous stem cell transplantation<br />

(mid 1990s) extended <strong>the</strong> <strong>the</strong>rapeutic options for this poor- risk<br />

subtype. Particularly <strong>the</strong> intensification <strong>of</strong> <strong>the</strong> chemo<strong>the</strong>rapeutic regimen<br />

and <strong>the</strong> combination with <strong>the</strong> monoclonal antibody Rituximab led to<br />

sustained disease control. We evaluated <strong>the</strong> efficacy <strong>of</strong> an intensified<br />

immunochemo<strong>the</strong>rapy combined with high-dose <strong>the</strong>rapy (HDT) and<br />

autologous stem cell rescue (ASCT) in patients with mantle cell lymphoma.<br />

Methods. Since 1995 14 out <strong>of</strong> 25 consecutive patients with newly<br />

diagnosed or pre-treated MCL <strong>of</strong> stage III-IV were eligible for HDT<br />

followed by autologous stem cell transplantation. Exclusion criteria for<br />

<strong>the</strong> remaining 11 patients were age over 70 (6), resistance to chemo<strong>the</strong>rapy<br />

(2), severe comorbidity (apoplexia (1), chronic atrial fibrillation(1))<br />

and recurrent infectious complications (tuberculosis (1)). Induction <strong>the</strong>rapy<br />

consisted <strong>of</strong> Rituximab (375 mg/m2 ; d1) plus CHOP-21 for 4-6 cycles<br />

followed by intensification (and priming) chemo<strong>the</strong>rapy for 2 to 3 cycles<br />

<strong>of</strong> CLAEG (3 days Cladribine 0,2 mg/kg, ARA- C 1,5 g/mÇ, Etoposid 60<br />

mg/m2 ) and on day 1 Daunoxome 80 mg/m2 or Idarubicine 8 mg/m2 .<br />

Autologous stem cell transplantation was performed at a median <strong>of</strong> 9 (7-<br />

13) months from diagnosis with 11 primary patients and 3 patients in<br />

relapsed disease status. In addition to high dose conditioning chemo<strong>the</strong>rapy<br />

(BEAM) 4 doses <strong>of</strong> Rituximab (d -9, -1, +48 and +55) were administered.<br />

Results. Fourteen Patients (6 female, 8 male) at a median age <strong>of</strong><br />

57 (49- 66) years were treated. 5 reached CR, 4 VGPR and 5 PR before<br />

receiving ASCT. In 4 patients Rituximab at day +48 and +55 was cancelled<br />

due to complications such as pneumonia (2), herpes zoster (1) or<br />

exan<strong>the</strong>ma (recurrent folliculitis/ ery<strong>the</strong>ma like lesions) (1). A median<br />

number <strong>of</strong> 5,64 ×106 /kg CD 34 positive cells (1,65-29,5) were reinfused.<br />

The engraftment was generally prompt and durable (granulocytes > 0,5<br />

G/l day +10 (9-11), platelets > 50 G/l day +14 (10-17)). Only in one case<br />

(male patient, age at TX: 55 years) a delayed regeneration (d +140; granulocytes<br />

> 0,5 G/L +11, platelets day +21) was observed. Side effects <strong>of</strong><br />

transplantation were mucositis grade I- II in 12 patients, 4 suffered from<br />

emesis grade II- III and 4 from enteritis grade I- II. Fever <strong>of</strong> unknown ori-<br />

12 th <strong>Congress</strong> <strong>of</strong> <strong>the</strong> <strong>European</strong> <strong>Hematology</strong> Association<br />

gin occurred in 4, septicaemia in 2 and pneumonia in 2. Grade 4 toxicities<br />

and treatment related deaths were not observed. Following transplantation<br />

all patients reached clinical and molecular CR. 12 patients are<br />

still in CCR with a median observation period <strong>of</strong> 33 months (5-58). 2<br />

patients relapsed after 17 and 26 months and died 58 and 34 months after<br />

transplantation. As late infectious complications 3 patients developed<br />

pneumonia and 3 herpes zoster. A propagation <strong>of</strong> ery<strong>the</strong>ma migrans<br />

and sarcoidosis was seen in 1 patient each. Conclusions. R- CHOP intensified<br />

by high dose treatment (CLAEG- D or CLAEG- Ida) including<br />

ASCT using BEAM regimen and Rituximab is accompanied by acceptable<br />

toxicity and improves outcome <strong>of</strong> patients with MCL, allowing<br />

long-term disease control.<br />

1260<br />

RESULTS OF CHILDHOOD ACUTE LYMPHOBLASTIC LEUKAEMIA TREATMENT USING<br />

EORTC 58881 AND EORTC 58951 PROTOCOLS<br />

M.A. Laatiri, F. Ben Moussa, I. Makhlouf, M. Bedoui, K. Zahra,<br />

M. Kortas, A. Khelif<br />

Farhat Hached Hospital, SOUSSE, Tunisia<br />

Childhood acute lymphoblastic leukemia (ALL) results have improved<br />

over time. The current results <strong>of</strong> <strong>the</strong> Department <strong>of</strong> <strong>Hematology</strong> in treating<br />

childhood ALL are being reported. From 1/1995 to 12/2004, 135 children<br />

(86 boys and 49 girls) with ALL have been treated with an ALL-<br />

EORTC 58881 (N=102 patients) or ALL-EORTC 58951 based protocol<br />

(since 1/2002, N=33 patients). Median age <strong>of</strong> diagnosis was 5,1 years<br />

(range, 1-15 years). The median WBC count at diagnosis was 22.3×109 /L<br />

(range 1.2×109 /L-1420.0×109 /L). Immunophenotype done in only 60<br />

patients revealed 45 B-cell precursor (40 CD10 + , 5 CD10 – ) and 15 T-cell<br />

ALL. Cytogenetic analysis was performed for 105 patients and revealed<br />

abnormalities in 47%. Persistent circulating leukemic blasts more than<br />

1.0×109 /L were present at day 8 in 22 patients (16%). Compared with<br />

<strong>the</strong> blast-negative group, <strong>the</strong>se patients had a significantly higher frequency<br />

<strong>of</strong> several adverse clinical features (WBC >50×109 /L, mediastinal mas,<br />

T-cell phenotype) and a significantly poorer prognosis (overall survival<br />

at 5 years 30% vs 70% for blast-negative group. Relapses were more frequent<br />

after EORTC-58881 particularly in blast-positive group. We conclude<br />

that <strong>the</strong> EORTC-based protocol as applied in our Department is<br />

tolerable with acceptable toxicity and that <strong>the</strong> modifications made to <strong>the</strong><br />

protocol by using EORTC 58951 improved <strong>the</strong> prognosis <strong>of</strong> patients<br />

with poor prognosis particularly T-cell leukaemia and blast-positive group.<br />

Longer follow-up is necessary to fur<strong>the</strong>r validate <strong>the</strong> results and credit<br />

<strong>of</strong> value <strong>the</strong> modifications made to <strong>the</strong> protocol.<br />

1261<br />

POST ASCT RITUXIMAB CONSOLIDATION THERAPY ELIMINATES PERSISTING FDG<br />

PET POSITIVITY IN DIFFUSE LARGE B CELL LYMPHOMA (DLBCL) PATIENTS<br />

P. Remenyi, 1 Szomor, 2 , . Bátai, 1 B. Kapás, 1 , A. Sipos, 1 Z. Csukly, 1<br />

M. Réti, 1 V. Goda1 , S. Lueff, 1 I. Bodó, 1 M.T. Masszi1 1 2 National Medical Center, BUDAPEST; University <strong>of</strong> Pécs I.Dept.<strong>of</strong> Medicine,<br />

PÉCS, Hungary<br />

Background. <strong>the</strong>re is evidence in <strong>the</strong> literature that combination <strong>of</strong> ASCT<br />

and rituximab is beneficial for DLBCL patients but <strong>the</strong> best treatment plan<br />

has not been established yet. Aims. to characterize post ASCT minimal<br />

residual disease by FDG PET scan and <strong>the</strong> effect <strong>of</strong> post ASCT rituximab<br />

consolidation <strong>the</strong>rapy on overall survival. Patients. between 04/2005-<br />

08/2006 16 consecutive DLBCL patients underwent autologous stem cell<br />

transplantation in two transplant centers . Transplant indication was<br />

relapse after conventional R-CHOP treatment, except for two pts who<br />

were transplanted in CR1 because <strong>of</strong> high risk disease. The median age<br />

was 50 years (19-60), female/male ratio was 8/8. All patients received rituximab<br />

containing salvage treatment (R-DHAP, RIME, R-GEMP) resulted<br />

in complete remission proved by CT scan in 12/16 cases. Conditioning regimen<br />

consisted <strong>of</strong> BEAM, <strong>the</strong> graft contained median 5.62×10 6 (3.77-11.41)<br />

CD34 + cells. One patient died <strong>of</strong> sepsis. FDG PET scan was performed two<br />

months after SCT. 3 months after SCT patients received standard dose<br />

(375 mg/m 2 ) rituximab in four consecutive weeks. Results. unfortunately 2<br />

patients relapsed within 2 months following SCT. Rituximab administration<br />

had no additive toxicity. 3/13 patients showed FDG PET positivity<br />

with low tumor burden indicating minimal residual disease. 3 months<br />

post rituximab treatment FDG PET scan turned to negative in all <strong>the</strong> previously<br />

positive (3/3) cases. 13/16 (81%) patients are in complete remission<br />

at a median 12 months (6-20) follow up time. Conclusions. post SCT<br />

rituximab may control low tumor burden without additive toxicity in<br />

DLBCL patients. Our data needs fur<strong>the</strong>r confirmation especially regard-<br />

haematologica/<strong>the</strong> hematology journal | 2007; 92(s1) | 457

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