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P-092 Chronic myelogenous monocytic leukemia with other tumors
Poster Presentations – 12th International Symposium on Myelodysplastic Syndromes / Leukemia Research 37 S1 (2013) S1–S117
(i) is constant (±6 months), (ii) does not meet the (minimal) criteria of MDS, and (iii) cannot be explained by any other haematological or non-haematological disease. Results: Of all 71 patients attended, 24%(17) were classified as ICUS, 65% (46) were clear diagnostic of MDS, 6% aplastic anemia, 4,5% (3) mieloproliferative syndrome and 1,5%(1) were mielocitic leukaemia. The cytogenetics was performed in only 23% (4) of patients with ICUS, not being evidenced no changes in karyotype. We found 88% (14) of them had unilineage, 12% (2) bilineage and 6% (1) trilineage cytopenias. The main alterations presented in bone marrow in this group were all all<10%: 100% (17) of diserytropoese, 29% (5) of disgranulopoese), 47% (8) of dismegariopoese. Conclusions: Initially we valorized the significance of morphologic in blood smears and histopathologic and tried to distinguish these entities only with these exams because they are more acessible. We point that morphology can be the center of diagnose and for us a very important guide to separate ICUS from MDS. In our casuistic we found that 24% of all patients attended were diagnosed as ICUS. The correct management and therapeutic considerations to this group are important due to possibility of MDS and AML development.
P-092 Chronic myelogenous monocytic leukemia with other tumors P. Wu, X. Du, J. Weng, P. Lai, L. Liu, H. Yao, J. Tong, S. Geng, X. Huang. Department of Haematology, Guangdong General Hospital/Guangdong Academiy of Medical Sciences, Guangzhou, China Background: Chronic myelomonocytic leukemia (CMML) is a rare clonal hematologic disorder, with a heterogeneous clinical and morphological expression, sharing features of both myelodysplastic syndromes (MDS) and chronic myeloproliferative disorders. Introduction: The association of CMML and lymphoma has been reported before, but other tumors is rare. In addition, few studies analyse the rate and the same features of CMML with other tumors systematically. Purpose: To report the clinical characteristics and treatment of patients with chronic myelomonocytic leukemia (CMML) and other tumors. Materials and Methods: A retrospective review of the medical records of patients with CMML and other tumors. Results: Based on our 37 CMML patients in recent four years, six cases (five male and one female, to be nearly 20%) are in comorbidity with other neoplasma, which respectively are two cases of nonHodgkin lymphoma (NHL), two cases of lung cancer, one case of nasopharyngneal carcinoma (NPC) and one case of esophageal carcinoma. Moreover, these people benefit less from both chemotherapy and bone marrow transplantation. Conclusions: As a result, it’s necessary to give the patients physical examination to find the very early stage solid tumors with CMML patients, in order to reduce misdiagnosis and underdiagnosis.
P-093 Hypoplastic myelodysplastic syndromes are not a specific clinical entity J. Schemenau 1 , S.E. Baldus 2 , M. Anlauf 2 , S. Blum 3 , K. Nachtkamp 4 , J. Neukirchen 4 , C. Strupp 4 , R. Haas 4 , N. Gattermann 4 , U. Germing 4 . 1 Department of Hematology and Oncology, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany; 2 Institute of Pathology, HeinrichHeine-University Düsseldorf, Düsseldorf, Germany; 3 Department of Hematology, University of Lausanne, Lausanne, Switzerland; 4 Department of Hematology and Oncology, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany Background: About 10-15% of all Myelodysplastic syndromes (MDS)
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present with hypocelluar bone marrow, but data on haematological characteristics and clinicial outcome are sparse. Introduction: Recently, Tong et al proposed a special prognostic scoring system for patients with MDS and hypocellular marrow. Purpose: The aim of our study was to describe haematological, cytogenetic, clinical, and prognostic characteristics of patients with hypocellular MDS using the data base of the Düsseldorf registry. Materials and Methods: We analysed data of 3742 patients, in which cellularity was assessed by means of central cytology and/or histology. Patients characteristics at the time of diagnosis were compared between hypercellular (N=1686), normocellular (n=1580) and hypocellular (n=476) cases. Prognostic parameters used in the IPSS as well as in the prognostic score for hypoplastic MDS were analysed. Patients were followed up until December 31th 2012. Results: There were no significant differences with regard to age, gender, haematological features, such as haemoglobin between hyper-, normo- and hypocellular cases (p=n.s.), whereas a significant difference in platelet levels between hypo- and normocellular MDS (p=0,005) and ANC levels between hypocellular compared to normoand hypercellular MDS (p=0,001) could be found. Differences with regard to cytogenetic risk groups, LDH, IPSS-, WPSSand IPSS-R risk groups, bone marrow blasts and general health condition could not be described (all p values n. s.), except for WHO Types (p=0,005). Median survival of the hypercellular cases was 20 months, as compared to 32 months in the normo-, and 29 months in the hypocellular cases (p=0,0005) The cumulative risk of AML evolution 2 and 5 years after diagnosis was comparable 29%, 20%, 25% after 2 years, 44%, 37%, 45% after 5 years (p=0,003). The risk groups defined by the recently published hypocellular prognostic score (Tong et al, Cancer 2012) differed in terms of prognosis, but the capacity of the score to subdivide the patients was even higher when applied to normo- and hypercellular cases. The low risk group of hypercellular patients had a worse prognosis as compared to normo- and hypocellular patients. Conclusions: Patients with hypocellular MDS did not differ significantly from hyper- and normocellular types with regard to haematopoietic insufficiency and prognosis and therefore should not define a separate MDS type. As the prognostic parameters did not differ, the hypocellular score is working fine, but is not specific. These cases can be prognosticated using the IPSS with the same results as compared to other MDS cases.
P-094 U2AF35 mutation is more prevalent in younger patients with myelodysplastic syndrome and associated with inferior outcomes S.J. Wu, J.L. Tang, H.F. Tien. Internal Medicine, National Taiwan University Hospital, Taipei city, Taiwan Background: The pathogenesis of myelodysplastic syndrome (MDS) has not been clearly identified. Introduction: Recurrent somatic mutation of U2AF35, one of the RNA splicing machinery genes, has been identified in a substantial proportion of MDS patients. Whether U2AF35-mutated patients have distinct clinical pictures or outcomes remains inconclusive. Purpose: We aimed to define the clinical correlations of U2AF35 mutation in MDS patients and examine the interactions of this mutation with other genetic alternations and the stability during disease progression. Materials and Methods: The bone marrow samples from a large MDS cohort was checked for the mutations of U2AF35 and other genes. U2AF35 mutation was correlated with the patients’ clinical characeteristics, concurrent genetic abberrations, and outcomes.
(i) is constant (±6 months), (ii) does not meet the (minimal) criteria of MDS, and (iii) cannot be explained by any other haematological or non-haematological disease. Results: Of all 71 patients attended, 24%(17) were classified as ICUS, 65% (46) were clear diagnostic of MDS, 6% aplastic anemia, 4,5% (3) mieloproliferative syndrome and 1,5%(1) were mielocitic leukaemia. The cytogenetics was performed in only 23% (4) of patients with ICUS, not being evidenced no changes in karyotype. We found 88% (14) of them had unilineage, 12% (2) bilineage and 6% (1) trilineage cytopenias. The main alterations presented in bone marrow in this group were all all<10%: 100% (17) of diserytropoese, 29% (5) of disgranulopoese), 47% (8) of dismegariopoese. Conclusions: Initially we valorized the significance of morphologic in blood smears and histopathologic and tried to distinguish these entities only with these exams because they are more acessible. We point that morphology can be the center of diagnose and for us a very important guide to separate ICUS from MDS. In our casuistic we found that 24% of all patients attended were diagnosed as ICUS. The correct management and therapeutic considerations to this group are important due to possibility of MDS and AML development.
P-092 Chronic myelogenous monocytic leukemia with other tumors P. Wu, X. Du, J. Weng, P. Lai, L. Liu, H. Yao, J. Tong, S. Geng, X. Huang. Department of Haematology, Guangdong General Hospital/Guangdong Academiy of Medical Sciences, Guangzhou, China Background: Chronic myelomonocytic leukemia (CMML) is a rare clonal hematologic disorder, with a heterogeneous clinical and morphological expression, sharing features of both myelodysplastic syndromes (MDS) and chronic myeloproliferative disorders. Introduction: The association of CMML and lymphoma has been reported before, but other tumors is rare. In addition, few studies analyse the rate and the same features of CMML with other tumors systematically. Purpose: To report the clinical characteristics and treatment of patients with chronic myelomonocytic leukemia (CMML) and other tumors. Materials and Methods: A retrospective review of the medical records of patients with CMML and other tumors. Results: Based on our 37 CMML patients in recent four years, six cases (five male and one female, to be nearly 20%) are in comorbidity with other neoplasma, which respectively are two cases of nonHodgkin lymphoma (NHL), two cases of lung cancer, one case of nasopharyngneal carcinoma (NPC) and one case of esophageal carcinoma. Moreover, these people benefit less from both chemotherapy and bone marrow transplantation. Conclusions: As a result, it’s necessary to give the patients physical examination to find the very early stage solid tumors with CMML patients, in order to reduce misdiagnosis and underdiagnosis.
P-093 Hypoplastic myelodysplastic syndromes are not a specific clinical entity J. Schemenau 1 , S.E. Baldus 2 , M. Anlauf 2 , S. Blum 3 , K. Nachtkamp 4 , J. Neukirchen 4 , C. Strupp 4 , R. Haas 4 , N. Gattermann 4 , U. Germing 4 . 1 Department of Hematology and Oncology, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany; 2 Institute of Pathology, HeinrichHeine-University Düsseldorf, Düsseldorf, Germany; 3 Department of Hematology, University of Lausanne, Lausanne, Switzerland; 4 Department of Hematology and Oncology, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany Background: About 10-15% of all Myelodysplastic syndromes (MDS)
S63
present with hypocelluar bone marrow, but data on haematological characteristics and clinicial outcome are sparse. Introduction: Recently, Tong et al proposed a special prognostic scoring system for patients with MDS and hypocellular marrow. Purpose: The aim of our study was to describe haematological, cytogenetic, clinical, and prognostic characteristics of patients with hypocellular MDS using the data base of the Düsseldorf registry. Materials and Methods: We analysed data of 3742 patients, in which cellularity was assessed by means of central cytology and/or histology. Patients characteristics at the time of diagnosis were compared between hypercellular (N=1686), normocellular (n=1580) and hypocellular (n=476) cases. Prognostic parameters used in the IPSS as well as in the prognostic score for hypoplastic MDS were analysed. Patients were followed up until December 31th 2012. Results: There were no significant differences with regard to age, gender, haematological features, such as haemoglobin between hyper-, normo- and hypocellular cases (p=n.s.), whereas a significant difference in platelet levels between hypo- and normocellular MDS (p=0,005) and ANC levels between hypocellular compared to normoand hypercellular MDS (p=0,001) could be found. Differences with regard to cytogenetic risk groups, LDH, IPSS-, WPSSand IPSS-R risk groups, bone marrow blasts and general health condition could not be described (all p values n. s.), except for WHO Types (p=0,005). Median survival of the hypercellular cases was 20 months, as compared to 32 months in the normo-, and 29 months in the hypocellular cases (p=0,0005) The cumulative risk of AML evolution 2 and 5 years after diagnosis was comparable 29%, 20%, 25% after 2 years, 44%, 37%, 45% after 5 years (p=0,003). The risk groups defined by the recently published hypocellular prognostic score (Tong et al, Cancer 2012) differed in terms of prognosis, but the capacity of the score to subdivide the patients was even higher when applied to normo- and hypercellular cases. The low risk group of hypercellular patients had a worse prognosis as compared to normo- and hypocellular patients. Conclusions: Patients with hypocellular MDS did not differ significantly from hyper- and normocellular types with regard to haematopoietic insufficiency and prognosis and therefore should not define a separate MDS type. As the prognostic parameters did not differ, the hypocellular score is working fine, but is not specific. These cases can be prognosticated using the IPSS with the same results as compared to other MDS cases.
P-094 U2AF35 mutation is more prevalent in younger patients with myelodysplastic syndrome and associated with inferior outcomes S.J. Wu, J.L. Tang, H.F. Tien. Internal Medicine, National Taiwan University Hospital, Taipei city, Taiwan Background: The pathogenesis of myelodysplastic syndrome (MDS) has not been clearly identified. Introduction: Recurrent somatic mutation of U2AF35, one of the RNA splicing machinery genes, has been identified in a substantial proportion of MDS patients. Whether U2AF35-mutated patients have distinct clinical pictures or outcomes remains inconclusive. Purpose: We aimed to define the clinical correlations of U2AF35 mutation in MDS patients and examine the interactions of this mutation with other genetic alternations and the stability during disease progression. Materials and Methods: The bone marrow samples from a large MDS cohort was checked for the mutations of U2AF35 and other genes. U2AF35 mutation was correlated with the patients’ clinical characeteristics, concurrent genetic abberrations, and outcomes.