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302 CLINICAL CHARACTERISTICS AND PROGNOSIS OF ATYPICAL CHRONIC MYELOID LEUKEMIA – REPORT BY POLISH ADULT LEUKEMIA GROUP
S150
Poster Presentations – 13th International Symposium on Myelodyspastic Syndromes / Leukemia Research 39 S1 (2015) S1–S166
Aims: Establishing approximately incidence in preschool children in Sarajevo and Bosnia and Herzegovina for period 1990 to 2014 for 50.000 preschool children avarage in Sarajevo Canton or 250.000 children avarage in Bosnia and Herzegovina. Methods: Tests that examine the blood and bone marrow areused to detect and diagnose myelodysplastic syndromes. Bone marrow aspiration and biopsy are very important. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow. Results: Mutations in splicing factors have been found in 60% of cases with myelodysplastic syndrome, particularly in those with ringed sideroblasts. The incidence is probably decreasing as the age of the population decreses increases, authors propose that the incidence in patients undre seven may be as high as 1.5 to 2 cases per 100,000 per year in last twenty five years. Conclusions: Early recognition of such problems and finding strategies to overcome them are important aspects of the comprehensive care of any child with a myelodysplastic syndromes. The goals of therapy are to control symptoms, improve quality of life, improve overall survival, and decrease progression to acute myelogenous leukemia. It is increasing incidence for preschool children in Bosnia specially in Sarajevo if comparing US or Western Europe incidence of MDS in younger children. 301 IRON OVERLOAD AND IRON CHELATION THERAPY WITH DEFERASIROX IN TRANSFUSION-DEPENDENT PATIENTS WITH MYELODYSPLASTIC SYNDROMES L. Del Corso1, A. Bacigalupo1, E. Balleari1, A. Bellodi1, T. Calzamiglia2, A. Da Col1, P. D’elia3, A. Dominietto1, S. Favorini1, G. Forni10, S. Galimberti4, M. Ghio1, R. Ghio1, C. Salvetti1, G. Ubezio1, L. Vignolo1, E. Molinari1, A. Ghiso1, M. Drousseau4, F. Simonetti5, R. Goretti6, R. Tassara7, O. Racchi8, M. Scudeletti9, E. Arboscello1, F. Gianluca10 1 hemato-oncology, IRCCS-AOU San Martino-Istituto Nazionale per la Ricerca sul Cancro, Genoa, Italy; 2Internal Medicine, Sanremo Hospital, Sanremo, Italy; 3immuno-hematology, Sant’Andrea Hospital, La Spezia, Italy; 4Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; 5Division of Medicine, Versilia Hospital, Camaiore, Italy; 6Internal Medicine, Pietra Ligure Hospital, Genoa, Italy; 7Internal Medicine, San Paolo Hospital, Savona, Italy; 8Hematooncology, Villa Scassi Hospital, Genoa, Italy; 9Internal Medicine, Sestri Levante Hospital, Sestri Levante, Italy; 10Hematology, Galliera Hospital, Genoa, Italy Background: Myelodysplastic syndromes (MDS) are one of the most common hematologic malignancies, the median age at diagnosis is 75 years in most of the series; therefore it can be considered a disease of the elderly patients (pts). Red blood cell transfusion (RBCT) is the main stone of supportive care and a cardinal option to keep patients alive while waiting for effects of specific therapeutic strategies. Transfusion dependency and the consequence iron overload (IOL) has been identified as an independent factor associated with decreased survival. The most important guidelines recommend to start iron chelation therapy (ICT) in all MDS pts with low- and INT1 risk disease with life expectancy >1 year, who have elevated serum ferritin (SF) up to 1000 mcg/L and/or received at least 20 RBCT. Methods: The safety and the efficacy were evaluated in a prospective, open label, single arm multicenter study of elderly transfusion-dependent “lower risk” MDS pts. Efficacy of ICT with DFX was examined by SF and when possible with imaging (MRI T2*). All pts received DFX at starting dose of 10 mg/kg/day increased up to 30 mg/kg/day according to transfusion regimen, SF, IOL, and tolerance. We evaluated hematological response, adverse events (AEs), drug interactions.
Results: Of 28 pts enrolled, 6 at screening visit presented hepatic IOL (4 RCMD, 2 RARS) evaluated by MRI; nobody presented cardiac IOL. In the subset of patients with IOL the median SF was 930 ng/mL, the median number of RBCT was 16 and over than 30% presented RARS. 12 pts completed 1 years of treatment, 4 died during the trial, 3 pts who discontinued DFX therapy: 1 due to progressive disease and 2 due to an AEs.The drug related AEs occurred in 10%. The most common side effects reported are non-progressive change in serum creatinine level and gastrointestinal disturbances. Significant risks for AEs were polypharmacotherapies and higher MDS-Specific Comorbidity Index. Median SF fell from 1358 mcg/L to 778 mcg/L after one year of treatment. The cumulative incidence of transfusion independence was 18%. The median time to transfusion independence was 5.4 months. Conclusion: IOL was presented also in pts with SF <1000 mcg/L and with number of RBCT < 20 units, particularly in pts with RARS. DFX therapy in MDS transfusion-dependent patients was tolerated and effectively lowered SF. Regular clinical multidisciplinary evaluation of comorbidity and concomitant therapy remains a critical components of successfully ICT. 302 CLINICAL CHARACTERISTICS AND PROGNOSIS OF ATYPICAL CHRONIC MYELOID LEUKEMIA – REPORT BY POLISH ADULT LEUKEMIA GROUP J. Drozd-Sokolowska1, K. Madry1, P. Szwedyk2, M. Razny2, G. Bober3, H. Krzemien3, M. Dutka4, A. Piekarska4, B. Stella-Holowiecka3, A. Waszczuk-Gajda1, H. Heleniak1, M. Paszkowska-Kowalewska1, J. Dwilewicz-Trojaczek1 1 Department of Hematology Oncology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland; 2Department of Hematology, Ludwik Rydygier Memorial Specialized Hospital, Cracow, Poland; 3Department of Hematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland; 4 Department of Hematology and Transplantology, Medical University of Gdansk, Gdansk, Poland Introduction: Atypical chronic myeloid leukemia (aCML) is a rare myelodysplastic/ myeloproliferative neoplasm overlap syndrome with a dismalprognosis. Little is known about its epidemiological characteristics. Treatment recommendations include cytoreductive agents and, possibly allogeneic stem cell transplantation, however the impact of these strategies on overall survival remains unclear. Materials and Methods: This report is a multicenter retrospective analysis of patients with aCML. The aim of the study was to determine the patients characteristics at diagnosis, as well as to evaluate used treatment strategies, transformation to acute leukemia, and overall survival. Results: The study group consisted of 23patients, 18 male (78.3%) and 5 (21.7%) female. The absence of BCR/ABL was confirmed by PCR (21/23 - 91.3%), or by FISH (12/23 – 52.2%), and solely by classical cytogenetics in 1 patient (4.35%). Cytogenetic analysis was performed for 13 patients (normal - 10, hypodiploid – 1, complex – 2). Median age at diagnosis was 66 years (range: 40-81). Physical abnormalities included splenomegaly (52.2%) and hepatomegaly (34.8%). Complete blood count parameters were as follows: white blood cell count – 70 G/l (20.9-342), hemoglobin concentration – 9 g/dl (3.9-14), platelet count – 69 G/l (31-492). All patients had elevated LDH activity. Initially 11 patients were transfusion dependent (47.8%). In the disease course 16 patients (69.6%) required red blood cell transfusion support. In 7 out of 9 patients (77%) initial ferritin concentration exceeded 1000 ng/ml, with only 4 of them being transfusion-dependant.
Poster Presentations – 13th International Symposium on Myelodyspastic Syndromes / Leukemia Research 39 S1 (2015) S1–S166
S151
Treatment consisted mostly of hydroxyurea (HU, 22/23). Other treatment options included mercaptopurine (4.3%), cytarabine (13%; either alone or in combination), and intensive chemotherapy (8.7%). 2 patients underwent allogeneic hematopoietic stem cell transplantation (alloHSCT) after myeloablative conditioning from related donors. Both obtained complete remission. 1 died due to GvHD, the other isalive after 44 months after alloHSCT. Median overall survival was 14.3 months (95% CI 6.2-16.6). Neither sex, age, splenomegaly, platelet count (<100 G/l vs ≥100G/l), percentage of immature progenitors in peripheral blood, transfusion dependence did effect OS. There was however a trend for a shorter OS for more severely anemic patients (< 9 g/dl vs ≥ 9 g/dl; p=0.07, log rank). 4 patients (17.4%) transformed to AML. Conclusions: aCML is a rare disease of mostly elderly, male patients. Prognosisis poor, with short median OS and high risk of AML transformation. Fit patients may benefit from allogeneic hematopoietic stem cell transplantation, which may offer cure in at least part of them.
Results: Among the cohort of 23 patients 20 met the MDS with isolated del (5q) WHO criteria and 3 were classified as refractory anemia with excess blasts (RAEB). The median age of the cohort was 80 years (range 60-94) composed of 16 females and 7 males. The main morphological features are as follows: At diagnosis neutropenia was observed in 8 patients (34%) (0.68-1.8x109/l) and thrombocytopenia in 5 patients (21%) (70-145x109/l). During the follow-up (1-62 months, median 22.6) 4 patients developed thrombocytopenia and severe neutropenia 5 to 14 months after diagnosis, these patients presented with dysgranulopoiesis. Conclusion: In this series we observed in addition to the most consistent feature of abnormal megakaryocytes, variable dysplasia including dysgranulopoiesis. The del (5q) syndrome is a low risk MDS but dysgranulopoiesis and thrombocytopenia have been previously identified as negative pronostic factors, this finding is confirmed in our study. These results underline the importance of the careful evaluation of dysplasia at the time of diagnosis.
303 CLINICAL AND MORPHOLOGICAL CHARACTERISTICS OF 23 PATIENTS WITH MYELODYSPLASIC SYNDROME ASSOCIATED WITH ISOLATED DEL(5Q) A. Eischen1, A. Ittel2, A.C. Galoisy1, C. Mayeur-Rousse1, L. Monier1, S. Ame3, C. Gervais2, C. Helias2, L. Mauvieux1 1 Laboratoire d’Hématologie, Hôpital de Hautepierre CHU de Strasbourg, Strasbourg Cedex, France; 2Laboratoire de Cytogénétique, Hôpital de Hautepierre CHU de Strasbourg, Strasbourg Cedex, France; 3Service d’Hématologie Clinique, Hôpital de Hautepierre CHU de Strasbourg, Strasbourg Cedex, France
304 EPIDEMIOLOGICAL AND PROGNOSTIC FACTORS FROM THE ARGENTINEAN MDS REGISTRY J. Gonzalez1, M. Flores1, J. Arbelbide1, M. Iabstrebner1, E. Nucifora1, A. Enrico2, V. Prates2, M. Rosenhain1, L. Koremblitt1, R. Crisp3, G. Alfonso3, A. Basquieras4, C. Martín2, I. Santos1, V. Barcalá1, C. Belli1 1 Hematology, Argentinean MDS Registry, CABA, Argentina; 2 Hematology, Argentinean MDS Registry, La Plata, Argentina; 3 Hematology, Argentinean MDS Registry, Haedo, Argentina; 4 Hematology, Argentinean MDS Registry, Cordoba, Argentina
Introduction: Myelodysplasic syndrome (MDS) with isolated del(5q) has been initially characterized by macrocytic anemia, morphological abnormalities of megakaryocytes and an interstitial deletion involving the long arm of chromosome 5. The current WHO classification requires blast count of <5% in the bone marrow (BM), <1% in the peripheral blood (PB) and the absence of Auer rods, but does not specify the morphology. Purpose: The aim of our study was to investigate the morphological characteristics of PB and BM in patients with MDS syndrome associated with del (5q). Material and methods: Between 2009 and 2014 23 patients were identified in our institution with MDS and isolated del(5q). Morphological evaluation of PB and BM was performed according to standard evaluation protocol. Dysplasia was recorded if the dysplasic features within one cell line constitued more than 10% of the cells.
Argentinean MDS Registry was created in 2008 and is sponsored by The Argentinean Society of Hematology. The main objective was to collect MDS related data to describe epidemiological characteristics, prognostic factors and scoring risk stratifications.
Table 1.
SCORE
n
Survival (months)
Time to AML (months)
Low Int-1 Int-2 High
157 148 48 23
105 44 17 13
116 31 9 4
Very-Low Low Intermediate High Very-High
110 151 41 40 31
105 NA 69 17 12
116 NA 12 10 9
Very-Low Low Intermediate High Very-High
25 142 74 63 22
105 NA 35 18 15
116 NA 32 12 6
Low Int-1 Int-2 High
103 175 84 80
NA 105 30 12
NA 116 11 9
0 1 2 3 ≥4
262 114 82 45 25
77 47 34 23 24
Risk Group
IPSS n:377
IPSS-R n:384
WPSS-R n:326
Laboratory and pathological features
Entire cohort n=23
RAEBI n=3
Hemoglobin (g per 100 ml) median and range
9.4 (7.4-11.8)
9.7 (8.0-10.8)
MCV (fl)
103 (92-129)
114 (101-123)
2.34 (0.68-3.96)
1.57 (1.37-1.80)
237 (71-435)
311 (210-435)
BM dyserythopoiesis number (%)
14 (60%)
3 (100%)
BM dysgranulopoiesis number (%)
11 (47%)
3 (100%)
BM dysmegakaryopoiesis (nonlobulated nuclei) number (%)
22 (96%)
3 (100%)
Trilineage dysplasia number (%)
9 (39%)
3 (100%)
Ring sideroblasts (>15%) number (%)
5 (21%)
0
1 RAEBII (5 months) 1 LAM (14 months)
1 RAEBII
Absolute neutrophil (count x 109/l) Platelets (count x 109/l)
Desease transformation (time in months from diagnosis)
Table 1.
MD Anderson Score n: 442
Charlson’s Comorbidities Index n: 528
Poster Presentations – 13th International Symposium on Myelodyspastic Syndromes / Leukemia Research 39 S1 (2015) S1–S166
Aims: Establishing approximately incidence in preschool children in Sarajevo and Bosnia and Herzegovina for period 1990 to 2014 for 50.000 preschool children avarage in Sarajevo Canton or 250.000 children avarage in Bosnia and Herzegovina. Methods: Tests that examine the blood and bone marrow areused to detect and diagnose myelodysplastic syndromes. Bone marrow aspiration and biopsy are very important. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow. Results: Mutations in splicing factors have been found in 60% of cases with myelodysplastic syndrome, particularly in those with ringed sideroblasts. The incidence is probably decreasing as the age of the population decreses increases, authors propose that the incidence in patients undre seven may be as high as 1.5 to 2 cases per 100,000 per year in last twenty five years. Conclusions: Early recognition of such problems and finding strategies to overcome them are important aspects of the comprehensive care of any child with a myelodysplastic syndromes. The goals of therapy are to control symptoms, improve quality of life, improve overall survival, and decrease progression to acute myelogenous leukemia. It is increasing incidence for preschool children in Bosnia specially in Sarajevo if comparing US or Western Europe incidence of MDS in younger children. 301 IRON OVERLOAD AND IRON CHELATION THERAPY WITH DEFERASIROX IN TRANSFUSION-DEPENDENT PATIENTS WITH MYELODYSPLASTIC SYNDROMES L. Del Corso1, A. Bacigalupo1, E. Balleari1, A. Bellodi1, T. Calzamiglia2, A. Da Col1, P. D’elia3, A. Dominietto1, S. Favorini1, G. Forni10, S. Galimberti4, M. Ghio1, R. Ghio1, C. Salvetti1, G. Ubezio1, L. Vignolo1, E. Molinari1, A. Ghiso1, M. Drousseau4, F. Simonetti5, R. Goretti6, R. Tassara7, O. Racchi8, M. Scudeletti9, E. Arboscello1, F. Gianluca10 1 hemato-oncology, IRCCS-AOU San Martino-Istituto Nazionale per la Ricerca sul Cancro, Genoa, Italy; 2Internal Medicine, Sanremo Hospital, Sanremo, Italy; 3immuno-hematology, Sant’Andrea Hospital, La Spezia, Italy; 4Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; 5Division of Medicine, Versilia Hospital, Camaiore, Italy; 6Internal Medicine, Pietra Ligure Hospital, Genoa, Italy; 7Internal Medicine, San Paolo Hospital, Savona, Italy; 8Hematooncology, Villa Scassi Hospital, Genoa, Italy; 9Internal Medicine, Sestri Levante Hospital, Sestri Levante, Italy; 10Hematology, Galliera Hospital, Genoa, Italy Background: Myelodysplastic syndromes (MDS) are one of the most common hematologic malignancies, the median age at diagnosis is 75 years in most of the series; therefore it can be considered a disease of the elderly patients (pts). Red blood cell transfusion (RBCT) is the main stone of supportive care and a cardinal option to keep patients alive while waiting for effects of specific therapeutic strategies. Transfusion dependency and the consequence iron overload (IOL) has been identified as an independent factor associated with decreased survival. The most important guidelines recommend to start iron chelation therapy (ICT) in all MDS pts with low- and INT1 risk disease with life expectancy >1 year, who have elevated serum ferritin (SF) up to 1000 mcg/L and/or received at least 20 RBCT. Methods: The safety and the efficacy were evaluated in a prospective, open label, single arm multicenter study of elderly transfusion-dependent “lower risk” MDS pts. Efficacy of ICT with DFX was examined by SF and when possible with imaging (MRI T2*). All pts received DFX at starting dose of 10 mg/kg/day increased up to 30 mg/kg/day according to transfusion regimen, SF, IOL, and tolerance. We evaluated hematological response, adverse events (AEs), drug interactions.
Results: Of 28 pts enrolled, 6 at screening visit presented hepatic IOL (4 RCMD, 2 RARS) evaluated by MRI; nobody presented cardiac IOL. In the subset of patients with IOL the median SF was 930 ng/mL, the median number of RBCT was 16 and over than 30% presented RARS. 12 pts completed 1 years of treatment, 4 died during the trial, 3 pts who discontinued DFX therapy: 1 due to progressive disease and 2 due to an AEs.The drug related AEs occurred in 10%. The most common side effects reported are non-progressive change in serum creatinine level and gastrointestinal disturbances. Significant risks for AEs were polypharmacotherapies and higher MDS-Specific Comorbidity Index. Median SF fell from 1358 mcg/L to 778 mcg/L after one year of treatment. The cumulative incidence of transfusion independence was 18%. The median time to transfusion independence was 5.4 months. Conclusion: IOL was presented also in pts with SF <1000 mcg/L and with number of RBCT < 20 units, particularly in pts with RARS. DFX therapy in MDS transfusion-dependent patients was tolerated and effectively lowered SF. Regular clinical multidisciplinary evaluation of comorbidity and concomitant therapy remains a critical components of successfully ICT. 302 CLINICAL CHARACTERISTICS AND PROGNOSIS OF ATYPICAL CHRONIC MYELOID LEUKEMIA – REPORT BY POLISH ADULT LEUKEMIA GROUP J. Drozd-Sokolowska1, K. Madry1, P. Szwedyk2, M. Razny2, G. Bober3, H. Krzemien3, M. Dutka4, A. Piekarska4, B. Stella-Holowiecka3, A. Waszczuk-Gajda1, H. Heleniak1, M. Paszkowska-Kowalewska1, J. Dwilewicz-Trojaczek1 1 Department of Hematology Oncology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland; 2Department of Hematology, Ludwik Rydygier Memorial Specialized Hospital, Cracow, Poland; 3Department of Hematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland; 4 Department of Hematology and Transplantology, Medical University of Gdansk, Gdansk, Poland Introduction: Atypical chronic myeloid leukemia (aCML) is a rare myelodysplastic/ myeloproliferative neoplasm overlap syndrome with a dismalprognosis. Little is known about its epidemiological characteristics. Treatment recommendations include cytoreductive agents and, possibly allogeneic stem cell transplantation, however the impact of these strategies on overall survival remains unclear. Materials and Methods: This report is a multicenter retrospective analysis of patients with aCML. The aim of the study was to determine the patients characteristics at diagnosis, as well as to evaluate used treatment strategies, transformation to acute leukemia, and overall survival. Results: The study group consisted of 23patients, 18 male (78.3%) and 5 (21.7%) female. The absence of BCR/ABL was confirmed by PCR (21/23 - 91.3%), or by FISH (12/23 – 52.2%), and solely by classical cytogenetics in 1 patient (4.35%). Cytogenetic analysis was performed for 13 patients (normal - 10, hypodiploid – 1, complex – 2). Median age at diagnosis was 66 years (range: 40-81). Physical abnormalities included splenomegaly (52.2%) and hepatomegaly (34.8%). Complete blood count parameters were as follows: white blood cell count – 70 G/l (20.9-342), hemoglobin concentration – 9 g/dl (3.9-14), platelet count – 69 G/l (31-492). All patients had elevated LDH activity. Initially 11 patients were transfusion dependent (47.8%). In the disease course 16 patients (69.6%) required red blood cell transfusion support. In 7 out of 9 patients (77%) initial ferritin concentration exceeded 1000 ng/ml, with only 4 of them being transfusion-dependant.
Poster Presentations – 13th International Symposium on Myelodyspastic Syndromes / Leukemia Research 39 S1 (2015) S1–S166
S151
Treatment consisted mostly of hydroxyurea (HU, 22/23). Other treatment options included mercaptopurine (4.3%), cytarabine (13%; either alone or in combination), and intensive chemotherapy (8.7%). 2 patients underwent allogeneic hematopoietic stem cell transplantation (alloHSCT) after myeloablative conditioning from related donors. Both obtained complete remission. 1 died due to GvHD, the other isalive after 44 months after alloHSCT. Median overall survival was 14.3 months (95% CI 6.2-16.6). Neither sex, age, splenomegaly, platelet count (<100 G/l vs ≥100G/l), percentage of immature progenitors in peripheral blood, transfusion dependence did effect OS. There was however a trend for a shorter OS for more severely anemic patients (< 9 g/dl vs ≥ 9 g/dl; p=0.07, log rank). 4 patients (17.4%) transformed to AML. Conclusions: aCML is a rare disease of mostly elderly, male patients. Prognosisis poor, with short median OS and high risk of AML transformation. Fit patients may benefit from allogeneic hematopoietic stem cell transplantation, which may offer cure in at least part of them.
Results: Among the cohort of 23 patients 20 met the MDS with isolated del (5q) WHO criteria and 3 were classified as refractory anemia with excess blasts (RAEB). The median age of the cohort was 80 years (range 60-94) composed of 16 females and 7 males. The main morphological features are as follows: At diagnosis neutropenia was observed in 8 patients (34%) (0.68-1.8x109/l) and thrombocytopenia in 5 patients (21%) (70-145x109/l). During the follow-up (1-62 months, median 22.6) 4 patients developed thrombocytopenia and severe neutropenia 5 to 14 months after diagnosis, these patients presented with dysgranulopoiesis. Conclusion: In this series we observed in addition to the most consistent feature of abnormal megakaryocytes, variable dysplasia including dysgranulopoiesis. The del (5q) syndrome is a low risk MDS but dysgranulopoiesis and thrombocytopenia have been previously identified as negative pronostic factors, this finding is confirmed in our study. These results underline the importance of the careful evaluation of dysplasia at the time of diagnosis.
303 CLINICAL AND MORPHOLOGICAL CHARACTERISTICS OF 23 PATIENTS WITH MYELODYSPLASIC SYNDROME ASSOCIATED WITH ISOLATED DEL(5Q) A. Eischen1, A. Ittel2, A.C. Galoisy1, C. Mayeur-Rousse1, L. Monier1, S. Ame3, C. Gervais2, C. Helias2, L. Mauvieux1 1 Laboratoire d’Hématologie, Hôpital de Hautepierre CHU de Strasbourg, Strasbourg Cedex, France; 2Laboratoire de Cytogénétique, Hôpital de Hautepierre CHU de Strasbourg, Strasbourg Cedex, France; 3Service d’Hématologie Clinique, Hôpital de Hautepierre CHU de Strasbourg, Strasbourg Cedex, France
304 EPIDEMIOLOGICAL AND PROGNOSTIC FACTORS FROM THE ARGENTINEAN MDS REGISTRY J. Gonzalez1, M. Flores1, J. Arbelbide1, M. Iabstrebner1, E. Nucifora1, A. Enrico2, V. Prates2, M. Rosenhain1, L. Koremblitt1, R. Crisp3, G. Alfonso3, A. Basquieras4, C. Martín2, I. Santos1, V. Barcalá1, C. Belli1 1 Hematology, Argentinean MDS Registry, CABA, Argentina; 2 Hematology, Argentinean MDS Registry, La Plata, Argentina; 3 Hematology, Argentinean MDS Registry, Haedo, Argentina; 4 Hematology, Argentinean MDS Registry, Cordoba, Argentina
Introduction: Myelodysplasic syndrome (MDS) with isolated del(5q) has been initially characterized by macrocytic anemia, morphological abnormalities of megakaryocytes and an interstitial deletion involving the long arm of chromosome 5. The current WHO classification requires blast count of <5% in the bone marrow (BM), <1% in the peripheral blood (PB) and the absence of Auer rods, but does not specify the morphology. Purpose: The aim of our study was to investigate the morphological characteristics of PB and BM in patients with MDS syndrome associated with del (5q). Material and methods: Between 2009 and 2014 23 patients were identified in our institution with MDS and isolated del(5q). Morphological evaluation of PB and BM was performed according to standard evaluation protocol. Dysplasia was recorded if the dysplasic features within one cell line constitued more than 10% of the cells.
Argentinean MDS Registry was created in 2008 and is sponsored by The Argentinean Society of Hematology. The main objective was to collect MDS related data to describe epidemiological characteristics, prognostic factors and scoring risk stratifications.
Table 1.
SCORE
n
Survival (months)
Time to AML (months)
Low Int-1 Int-2 High
157 148 48 23
105 44 17 13
116 31 9 4
Very-Low Low Intermediate High Very-High
110 151 41 40 31
105 NA 69 17 12
116 NA 12 10 9
Very-Low Low Intermediate High Very-High
25 142 74 63 22
105 NA 35 18 15
116 NA 32 12 6
Low Int-1 Int-2 High
103 175 84 80
NA 105 30 12
NA 116 11 9
0 1 2 3 ≥4
262 114 82 45 25
77 47 34 23 24
Risk Group
IPSS n:377
IPSS-R n:384
WPSS-R n:326
Laboratory and pathological features
Entire cohort n=23
RAEBI n=3
Hemoglobin (g per 100 ml) median and range
9.4 (7.4-11.8)
9.7 (8.0-10.8)
MCV (fl)
103 (92-129)
114 (101-123)
2.34 (0.68-3.96)
1.57 (1.37-1.80)
237 (71-435)
311 (210-435)
BM dyserythopoiesis number (%)
14 (60%)
3 (100%)
BM dysgranulopoiesis number (%)
11 (47%)
3 (100%)
BM dysmegakaryopoiesis (nonlobulated nuclei) number (%)
22 (96%)
3 (100%)
Trilineage dysplasia number (%)
9 (39%)
3 (100%)
Ring sideroblasts (>15%) number (%)
5 (21%)
0
1 RAEBII (5 months) 1 LAM (14 months)
1 RAEBII
Absolute neutrophil (count x 109/l) Platelets (count x 109/l)
Desease transformation (time in months from diagnosis)
Table 1.
MD Anderson Score n: 442
Charlson’s Comorbidities Index n: 528