Haematologica 2004;89: supplement no. 6 - Supplements ...

VIII Congress of the Italian Society of Experimental Hematology, Pavia, September 14-16, 2004133ian Association for Cancer Research (A. I. R. C. ), by theItalian National Research Council (C. N. R), and bygrants from the Campania Region, Fondazione delMonte di Bologna e Ravenna and A. I. L.PO-117CLINICAL-HEMATOLOGICAL FINDINGS AND MOLECULARRESULTS IN A NEW SERIES OF CHRONIC EOSINOPHILICLEUKEMIA WITH 4Q12 CRYPTIC DELETIONLa Starza R,* Crescenzi B,* Beacci D,* Gurdo G,*Specchia G, # Nozzoli C, + Foppoli M, § Luciano L,°Matteucci C,* Martelli MF,* Marynen P, § Mecucci C**Ematologia, Policlinico Monteluce, Università degliStudi di Perugia; # Hematology, University of Foggia,Italy; + Hematology, University of Firenze, Italy;§Ospedale San Raffaele, Milano, Italy; °Hematology,University ofNapoli, Italy; § Centre for Human Geneticsand VIB, University of Leuven, BelgiumAccording to WHO criteria, chronic eosinophilicleukemia (CEL) represent myeloproliferative disorderswith increased eosinophils in the peripheral blood(>1.500/mL) lasting more than six months, exsclusionof secondary causes, presence of signs or symptomsof organ involvement, and demonstration of clonalityof eosinophils or increased bone marrow blasts.We report on clinical-hematological and molecularfindings in ten patients with 4q12-/CEL. Cytogeneticswas done on bone marrow cells after culturing24/48 hours. Metaphases were G-banded withWright stain and karyotypes were described accordingto the International System for Human CytogeneticNomenclature (1995). Interphase-FISH was performedwith a set of probes, RP11-120K16, RP11-3H20(kindly provided by Dr P Marynen, University ofLeuven, Belgium), designed to detect 4q12 crypticdeletion which underlies the FIP1L1/PDGFRα rearrangement.In three patients PCR investigationsconfirmed the FIP1L1/PDGFRα fusion protein.Patients were nine males and one female agedbetween 29 and 68.All patients had an hyperleukocytosis(7000-129000/L) with increased eosinophils(range of absolute number: 2088-30048). Increasedbone marrow eosinophils were documented in 5/6patients with available bone marrow biopsy. Splenomegalywas present in 8/10 while hepatomegaly waspresent in all 7 analysed cases. Other signs or symptomsof organ involvement were present in 7/10patients with the following distribution: hearth 3 cases,skin 3 cases, lung 2 cases, central nervous system2 cases. Karyotypes were normal in 7/10 analysedpatients. The cells bearing deletion of clone RP11-3H20 ranged from 43% to 96% at diagnosis. Treatmentwith imatinib mesylate induced rapid hematologicalremission in 7/7 patients. FISH and PCR arecompared in the monitoring of molecular remission.Funding: This work was partially supported by CNR-MIUR and FIRB.PO-118ANTI CD-20 TREATMENT HALTED PROGRESSION OF THEHAEMOLYTIC PROCESS IN MYELOFIBROSIS WITH MYELOIDMETAPLASIA PATIENT (MMM)AND SEVERE TRANSFUSIONDEPENDENT ANEMIA. A CASE REPORTIuliano F, Fabiano F, Mannella A, Rizzo C, Viscomi L,Peta AU. O. di Ematologia. Azienda Ospedaliera"Pugliese-Ciaccio", Catanzaro, ItalySome reports suggest that immunologic mechanismmay play a role in the pathogenesis of anemia inmyelofibrosis with myeloid metaplasia (MMM). Moreover,rituximab,a chimeric monoclonal antibodyagainst CD-20, has been increasingly recognised as auseful therapeutic agent for immuno-mediated disorders.We report of a patient suffering from MMM andsevere transfusion dependent anemia in wich anti CD-20 treatment halted progression of the haemolyticprocess. A 70-year-old male was diagnosed on January1997 as MMM low risk,pathologic stage II withnormal karyotype. Diabetes and paroxysmal atrial fibrillationwere co-morbidities. Because of thrombocytosisand abdominal discomfort due to liver and spleenenlargement, hydrea was given at dose of 20 mg/Kgthree times weekly. Treatment with danazol 200mg/daily, rHuEPO 10000 U scx3 weekly and folic acidwas added 5 years later, when Hb level dropped below90 g/L. Nevertheless a median of 2 packaged red bloodcell units was needed monthly. Increased serum levelsof unconjugated bilirubin and LDH,a slightly reductionin serum haptoglobin and C3c along with a mild reticulocytosis,were sugestive of hemolytic anemia, despiteDAT negative test (IgG, IgA, C3d), CD55, CD59 normalflow cytometric assay, normal GP6D level and absenceof cold agglutinins in the plasma. According to publisheddata concerning the cyclosporine-A effects inimproving anemia in MMM, a cyclophosphamide doseof 100 mg/daily was performed. The patient becametransfusion free in 5 months and he did not need anytransfusion for one year long. At the time of relapse,EDX was stopped and after three months of azatioprinetreatment,anemia progressively worsened and thepatient became heavily transfusion dependent. Keepingin mind his clinical history,we decided to treat thepatient with rituximab to inhibit the underlyingimmunologic mechanism. Rituximab was given in a500 mg total dose, once weekly on 4 consecutivetimes. Response to treatment was evaluated by theimprovement of the parameters of hemolysis, such asdecrease in bilirubin and LDH, increase in Hb levelshaematologica vol. 89[suppl. n. 6]:september 2004