Abstract
6686 Background: Acute myeloid leukemias (AML) with near-tetraploidy (NT: 80–104 chromosomes, without aberrant near-diploid metaphases) were described in about 35 patients. The aim of the study was to collect laboratory and clinical data of patients with these rare types of AML. Methods: We describe 29 cases with NT-AML collected by the EGIL in a retrospective multicenter study from 8 European countries diagnosed between 1984 and 2003. Results: Three NT-AML cases were misdiagnosed as lymphoblastic malignancies. Imunophenotyping and liquid cultures of blasts with a phorbol TPA was essential for diagnosis in 13 and 2 cases, respectively. Twenty-three cases with M0-M5 AML (12 M0, 3 M1, 2 M2, 2 M4, 3 M5, 1 NOS), 19–83 (median 64) years old, exhibited erythroid and/or megakaryocytic (EM) dysplasia, CD34+ and other similarities and may represent a category of multi-lineage NT-AML M0-M5. Multi-lineage involvement was also found in 3 M6 and 1 M7 cases. Ten females, 36–83 (median 70.5) years old, were significantly older (p=0.031) than 17 males, 19–83 (median 59) years old. In 10 cases older than 60 years we diagnosed a secondary NT-AML that developed after chemo-and/or radiotherapy or after 2–7 months of a myelodysplasia. Complete remission (CR) was reached in 6 of 9 cases with M0-M5 AML younger than 60 years treated with intensive cytosine arabinoside and an anthracycline based therapy. Their median survival was 25.3 months with 3 cases having been at their 1st continuous CR after autologous stem cell transplantation (ASCT) for 21–54 months demonstrating the efficacy of this treatment modality. Only 3 of 7 cases older than 60 years with de novo M0-M5 AML reached CR and survived 15–32 months. None of cases with secondary M0-M5 AML reached CR. Their survival was short as that of M6 and M7 cases. Another category may represent a 67-year old patient with AML M0 without EM-dysplasia (single-lineage AML) who survived 80 months after standard dose chemotherapy. Conclusions: Among NT-AML patients the worst prognosis exhibit those with secondary multi-lineage M0-M5, M6, M7; a somewhat better prognosis cases with de novo multi-lineage M0-M5, especially after ASCT at the 1st CR. The best prognosis may exhibit patients with single-lineage NT-AML. No significant financial relationships to disclose.
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