- Email: [email protected]
P-67 Hyperdiploidy in MDS and AML is associated with poor prognosis
Genetics megakaryocytes with multiple small nuclei. BM biopsy was hypercellular with trilineage dysplasia and presented ALIPS. BM karyotype showed: 47, XY, +8[20]. The diagnosis was RAEB-1 (WHO) and IPSS was intermediate-2. Discussion: PA defects are common in MDS, but consequent bleeding is not. In the case here presented purpuras were common and recurrent. We believe that these PA defects could be associated with auto immune phenomena (AI). Besides purpura, the patient developed intense arthralgia in hands, knees and shoulders as seen in rheumatoid patients. Trisomy 8 has been related to AI as vasculitis and Behcet's syndrome. In conclusion, MDS patients should be more carefully studied for autoimmune phenomena.
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significant survival advantage (p 0.0439 and p 0.0171, respectively). Conclusions: We showed the typical morphologic features, clinical characters and outcomes of MDS patients with der(1 ;7) in Japan. We highlighted the impact of blast percentage and additional chromosome abnormalities on survival in these patients.
[P~
HYPERDIPLOIDY IN MDS AND AML IS ASSOCIATED WITH POOR PROGNOSIS
S. Balleisen1, S. Knipp 1 *, A. Kuendgen 1, B. Hildebrandt2, N. Gattermann 1, R. Haas 1, B. Royer-Pokora 2, U. Germing1. 1Haematology, University Dusseldorf
2Human Genetics University Dusseldorf Germany *E-maih [email protected] f.de CHARACTERISTICS OF MYELODYSPLASTIC SYNDROMES WITH der(1;7)(ql0;pl0): REPORT OF 19 CASES FROM JAPAN
H.-H. Hsiao 1 *, K. Ohyashiki2, G. Sashida 2, Y. Ito2, A. Kodama 3, Y.-C. Liu1, S.-E Lin 1. 1Division of
Hematology-Oncology, Department of Internal Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; 2The First Department of Internal Medicine, 3Central Laboratory (Chromosome UniO, Tokyo Medical University, Tokyo, Japan *E-maih [email protected] Background: The authors reported myelodysplastic syndromes (MDS) with der(1;7)(ql0;pl0) [hereafter der(1;7)] abnormality in Japan and reviewed their clinical characteristics and outcomes. Methods: From 1993 to 2004, 19 MDS patients with der(1;7) was noted in Tokyo Medical University hospital and enrolled in the study retrospectively. Students' t-test, chi-square and Kaplan Meier method were used for statistics. Results: The der(1;7) showed overwhelming male predominance (male: female 19:0). Cytopenia in peripheral blood examination and trilineage dysplasia pictures were the typical presentation of patients, i.e., refractory cytopenia with multilineage dysplasia subtype in the new WHO classification. About 26.3% of patients had a history of genotoxic exposure (therapy related MDS), especially radiation and antimetabolites, due to preceding solid tumors. The history of genotoxic exposure was significantly associated with the additional chromosome changes (p 0.0466). The outcome was poor with short median survival (406 days) and high transformation rate (40%). There was no significant difference in survival in terms of genotoxic exposure, white cell count level, hemoglobin level, platelet count level, age and percentage of der(1;7). Only low blast percentages and sole der(1;7) anomaly had
In order to determine the frequency and prognostic impact of hyperdiploidy, we reviewed 332 cases of acute myeloid leukemia (AML) and 718 cases ofmyelodysplastic syndrome (MDS) that were diagnosed, karyotyped and followed up at our institutions. Hyperdiploidy was present in 4 of 332 (1.2%) patients with AML. Three had a massive hyperdiploidy (50, 54, and 77 chromosomes, respectively). Two of them achieved CR, but both relapsed within three months. None of the patients is alive, and median survival was 5 months. A hyperdiploid karyotype was documented in 12 of 718 (1.7%) patients with MDS (4 × RA, 8 × RAEB or RAEB-T). Seven patients had a massive hyperdiploidy (median 52, range 50 93 chromosomes). In 4 patients, the disease showed progression to AML. Among 5 patients who received induction chemotherapy 3 achieved CR but had an early relapse. Median survival in MDS patients with hyperdiploidy was 10 months (5 18). Hyperdiploidy is a complex karyotype, which is an important adverse risk factor in MDS and AML. In MDS, we are not aware of any previous investigations addressing this issue. Hyperdiploidy was rare in our patients with AML and MDS, but clearly associated with a poor outcome. In summary, we found that hyperdiploidy is an unfavorable prognostic factor in adult patients with hematological malignancies such as AML, and MDS.
~RARE CHROMOSOMAL ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES D.S. Kyriakou 1, A.-V.S. Karathoda 1 *, A. Tsezou 2, G. Karras 1, K. Karakousis 1, N. Stathakis 1. 1Department
of Hematology University Ho~Tital of Thessalia, 2Department of Medical Genetics University Ho~Tital of Thessalia, Greece *E-maih [email protected] Introduction: The MDS are often characterized by chromosomal abnormalities. In 50% of all cases chromosomes
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significant survival advantage (p 0.0439 and p 0.0171, respectively). Conclusions: We showed the typical morphologic features, clinical characters and outcomes of MDS patients with der(1 ;7) in Japan. We highlighted the impact of blast percentage and additional chromosome abnormalities on survival in these patients.
[P~
HYPERDIPLOIDY IN MDS AND AML IS ASSOCIATED WITH POOR PROGNOSIS
S. Balleisen1, S. Knipp 1 *, A. Kuendgen 1, B. Hildebrandt2, N. Gattermann 1, R. Haas 1, B. Royer-Pokora 2, U. Germing1. 1Haematology, University Dusseldorf
2Human Genetics University Dusseldorf Germany *E-maih [email protected] f.de CHARACTERISTICS OF MYELODYSPLASTIC SYNDROMES WITH der(1;7)(ql0;pl0): REPORT OF 19 CASES FROM JAPAN
H.-H. Hsiao 1 *, K. Ohyashiki2, G. Sashida 2, Y. Ito2, A. Kodama 3, Y.-C. Liu1, S.-E Lin 1. 1Division of
Hematology-Oncology, Department of Internal Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; 2The First Department of Internal Medicine, 3Central Laboratory (Chromosome UniO, Tokyo Medical University, Tokyo, Japan *E-maih [email protected] Background: The authors reported myelodysplastic syndromes (MDS) with der(1;7)(ql0;pl0) [hereafter der(1;7)] abnormality in Japan and reviewed their clinical characteristics and outcomes. Methods: From 1993 to 2004, 19 MDS patients with der(1;7) was noted in Tokyo Medical University hospital and enrolled in the study retrospectively. Students' t-test, chi-square and Kaplan Meier method were used for statistics. Results: The der(1;7) showed overwhelming male predominance (male: female 19:0). Cytopenia in peripheral blood examination and trilineage dysplasia pictures were the typical presentation of patients, i.e., refractory cytopenia with multilineage dysplasia subtype in the new WHO classification. About 26.3% of patients had a history of genotoxic exposure (therapy related MDS), especially radiation and antimetabolites, due to preceding solid tumors. The history of genotoxic exposure was significantly associated with the additional chromosome changes (p 0.0466). The outcome was poor with short median survival (406 days) and high transformation rate (40%). There was no significant difference in survival in terms of genotoxic exposure, white cell count level, hemoglobin level, platelet count level, age and percentage of der(1;7). Only low blast percentages and sole der(1;7) anomaly had
In order to determine the frequency and prognostic impact of hyperdiploidy, we reviewed 332 cases of acute myeloid leukemia (AML) and 718 cases ofmyelodysplastic syndrome (MDS) that were diagnosed, karyotyped and followed up at our institutions. Hyperdiploidy was present in 4 of 332 (1.2%) patients with AML. Three had a massive hyperdiploidy (50, 54, and 77 chromosomes, respectively). Two of them achieved CR, but both relapsed within three months. None of the patients is alive, and median survival was 5 months. A hyperdiploid karyotype was documented in 12 of 718 (1.7%) patients with MDS (4 × RA, 8 × RAEB or RAEB-T). Seven patients had a massive hyperdiploidy (median 52, range 50 93 chromosomes). In 4 patients, the disease showed progression to AML. Among 5 patients who received induction chemotherapy 3 achieved CR but had an early relapse. Median survival in MDS patients with hyperdiploidy was 10 months (5 18). Hyperdiploidy is a complex karyotype, which is an important adverse risk factor in MDS and AML. In MDS, we are not aware of any previous investigations addressing this issue. Hyperdiploidy was rare in our patients with AML and MDS, but clearly associated with a poor outcome. In summary, we found that hyperdiploidy is an unfavorable prognostic factor in adult patients with hematological malignancies such as AML, and MDS.
~RARE CHROMOSOMAL ABNORMALITIES IN MYELODYSPLASTIC SYNDROMES D.S. Kyriakou 1, A.-V.S. Karathoda 1 *, A. Tsezou 2, G. Karras 1, K. Karakousis 1, N. Stathakis 1. 1Department
of Hematology University Ho~Tital of Thessalia, 2Department of Medical Genetics University Ho~Tital of Thessalia, Greece *E-maih [email protected] Introduction: The MDS are often characterized by chromosomal abnormalities. In 50% of all cases chromosomes