S98 Poster Presentations – 12th International Symposium on Myelodysplastic Syndromes / Leukemia Research 37 S1 (2013) S1–S117

marrow failure, who presented with paraneoplastic inflamma-tory/autoimmune disease. Illustrated characterization of the clinicalphenotype will be presented. Current concepts of MDS pathophysiol-ogy and treatment in light of aberrant/paraneoplastic inflammationwill be discussed.Conclusions: As paraneoplastic syndroms are often mistakenly diag-nosed as idiopathic autoimmune disorders, we propose that coexis-tence of an underlying myelodysplastic syndrome should be consid-ered early in the diagnostic work-up. This requires close interactionbetween haematologist and rheumatologist.

P-165Erythropoietic aspects in MDS patients responders to ESAstreatment

P. Danise1, O. Vitagliano2, C. Cerchione2, I. Soriente1, A.M. D’Arco1,F. Pane2, F. Alfinito2. 1Oncology/Hematology, Umberto I Hospital,Nocera Inferiore (SA), Italy; 2Hematology, University of Naples FedericoII, Naples, Italy

Background: ESAs are the first line therapy in low risk anemic MDSpatients and an early inception of this therapy can delay the need forRBC transfusion, hypothetically by slowing the disease course.Introduction: It is a matter of debate whether the clinical responseis a result ofproliferation and maturation of the dysplastic clone orstimulation ofresidual normal erythropoiesis by ESAs. Evidence hasbeen provided tosupport both views.Purpose: Macrocytosis is one of the cytological hallmarks ofdyserithropoiesis in MDS: in this work we have analyzed erythropoi-etic response to ESAs therapy in a cohort of anemic MDS prevalently“low risk” patients enrolled in a regional retrospective register, RE-CAMDS (Registro Campano Mielodisplasie), subgroup of the ItalianMDS register. We focused on cytometric differences in Mean Corpus-colar Volume of erythrocytes during the observation period in orderto speculate on the target of such therapy in responsive patients.Materials and Methods: 97 anemic MDS patients (41 RA, 16 RARS,35 RCMD, 5 RAEB), not transfusion dependent, under standard ESAtreatment (α and β Epo, 40000/80000 or 30000/60000 U/weekly re-spectively), were analyzed at the baseline, after three and six monthof continuous therapy. The response rate was evaluated followingIWG criteria.Statistical analysis was performed with χ2 and Anova tests.Results: ESA therapy was started at Hb concentration 9g/dl±1,06,global response rate was 84% (81/97), no difference amongWHO sub-groups was found. 74 patients responded after 3 months, 7 after 6.In the responsive cohort, MCV was higher than normal at baselinein 43/81 (53%) patients, while 12/16 (80%) non-responsive patientsexhibited macrocytosis.During the response at ESAs treatment, 35/43 (81%) macrocytic pa-tients showed permanently elevated values of MCV whereas 8 (18%)macrocytic responsive patients became permanently normocytic. Inthe group of 38 initially normocytic responsive patients 8 (21%) be-came macrocytic and contemporarily 4 of them showed an increasein their neutropenia and/or thrombocytopenia.Conclusions: These very preliminary data can suggest that in thema-jority of MDS patients responsive to ESA treatment the increase ofhemoglobin level occurs mainly stimulating erythroid production inMDS clones; in the minority of patients probably it happens recruit-ing residual polyclonal erythropoiesis. It is interesting to note thatstimulating effects of ESA last even when the expression of dysplasiaprogresses.

P-166Disability and disease burden in outpatient myelodisplasticsyndromes

A. Tendas, L. Cupelli, M. Giovannini, G. Catalano, L. Scaramucci,A. Perrotti, P. Niscola, P. De fabritiis. Hematology, S.Eugenio Hospital,Rome, Italy

Background: Patients (pts) with myelodysplastic syndromes (MDS),as an effect of MDS, comorbidities and complications (“disease bur-den”), may develop different grade of functional impairment, such asmotor function impairment (MFI), till activities of daily living (ADL)interference.Introduction: Literature data are scarce.Purpose: To address MFI frequency, cause and relation with diseaseburden.Materials and Methods: Cross-sectional evaluation in consecutiveMDS patients. MFI was assessed as reported in Table 1. MFI mecha-nisms were explored by addressing disease burden: organ diseaseswere scored as 0 (no disease), 1 (present disease, without MFI), 2(present disease, inducing fine-MFI), 3 (present disease, inducingbasic-MFI); etiology was defined.Results: There were 27 pts (16 male); median age 70 (49- 81) years.Median interval-from-diagnosis was 1.2 years (range 0.04-12.63). Atdiagnosis, according to IPSS (complete in 25 and incomplete in 2cases), risk was: low, intermediate-1, intermediate-2, high and unde-termined in 5, 11, 7, 2 and 2 pts, respectively. At study time, diseasestatus was: 25 chronic phase and 2 complete remission; 8 patientswere on active treatment (azacitidine 6, lenalidomide 1, allogeneictransplantation 1) and 19 on supportive treatment only. Basic-MFI(BI<100%) was present in 2/27 (7.4%) pts; disabilitywas moderate onboth pts. f-MD (RMI= 12-14) was present in 5/27 (18.5%) pts; overall,MFI was present in 7/27 (26%) MDS pts. Thirty organ diseases wereidentified among 16/27 (59%) pts, ranging from 1 to 8 diseases perpt; organ diseases were classified as: 25 comorbidities (median score=1), 2 therapy-related complications (median score =2) and 3 MDScomplications (median score =1); curability was addressed as: possi-ble, doubt or absent in 16, 2 and 12 diseases, respectively. Target or-gan/apparatus was: bone/joint 6, cardiovascular 6, muscle 6, nervoussystem 4, endocrine system 4, eye 3, kidney 1, lung 1, urinary tract 1.Among 7 MFI pts, 19 organ diseases were identified (median 2; range1-8); 14 comorbidities, 2 therapy-related complications and 3 MDScomplications; 11/19 diseases, among MFI pts, were MFI-inducingdiseases: 7 comorbidities, 2 t-rel complications and 2 MDS compli-cations (median 1.5 (range 1-4) diseases per pt); curability was: pos-sible, doubt or absent in 6, 1 and 4 diseases, respectively.

Table 1

Step 1. Barthel Index calculation100 66–99 33–65 0–32

Basic-MFI Absent c. Mild-MFI d. Moderate-MFI e. Severe-MFI

Step 2. RMI calculationRMI = 15 RMI = 12–14

MFI a. No MFI b. Fine-MFI

MFI, Motor function impairment; B-MFI, basic-MFI; RMI, Rivermead Motility Index.

Conclusions:MFI and disease burden are relevant issues amongMDSpts.

P-167New risk group staging according to new R-IPSS of 25 patientswith low-risk MDS

A. Tatic1, C. Jardan1, O. Georgescu1, O. Stanca2, M. Vasilica1,S. Badelita1, A.M. Crisan1, A. Colita1, D. Colita1, G. Vulcan3,A.R. Lupu2, D. Coriu1. 1Hematology and Bone Marrow Transplant,“Prof. Dr. Stefan Berceanu” Department, Fundeni Clinical Institute,