Unusually Long Survival of a 67-Year-Old Patient with Near-Tetraploid Acute Myeloid Leukemia M0 without Erythroblastic and Megakaryocytic Dysplasia

Lemez, P; Klamová, Hana; Zemanová, Zuzana; Marinov, Iuri; Fuchs, Ota; Schwarz, Jiřı́; Březinová, Jana; Provazníková, Dana; Kostecka, Arnost; Marková, Jana; Michalová, K; Jelı́nek, Jaroslav

doi:10.1159/000328199

Cited by 6 publications

(5 citation statements)

References 48 publications

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“…Moreover, the patient was a young man with good underlying conditions so possibly these issues are an important point in the long survival of our patient. In the literature, there are also other near-tetraploidy or tetraploidy AML reported cases with unusually long survival 10. In this case report for example, the authors highlight the absence of dysplasia at diagnosis as the main reason of a better overall survival.…”

Section: Discussionmentioning

confidence: 65%

Tetraploidy acute myeloid leukaemia after chromosome 16 inversion

et al. 2017

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Our patient is a 36-year-old man referred by his general physician to the Department of Hematology because of mild neutropenia in a routine analysis at work. There was no history of previous diseases, and examination was normal. Blood investigations confirmed the neutropenia and showed elongation of prothrombin time. A bone marrow examination was performed revealing about 10% of myeloblasts on the aspirate smears. A cytogenetic study showed chromosome 16 inversion in all of these cells and tetraploidy only in some of them, which were extremely large in size. According to the revised WHO classification of tumours (2008), the patient was diagnosed as a case of acute myeloid leukaemia with chromosome 16 inversion.

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Section: Discussionmentioning

confidence: 65%

Tetraploidy acute myeloid leukaemia after chromosome 16 inversion

et al. 2017

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“…Although the patient received only one course of remission induction chemotherapy due to severe comorbidities, he remained in complete remission for more than 40 months without any consolidation or maintenance therapies. Lemez et al also reported a case of NT-AML with unusually long survival (80 months) (9). Some cases of NT/T-AML might have a better prognosis than generally expected.…”

Section: Discussionmentioning

confidence: 88%

Near Tetraploidy Acute Myeloid Leukemia in Long-term Remission with Persistent Clonal Hematopoiesis with del(20)(q12q13)

et al. 2018

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Abstract:Patients with near tetraploidy/tetraploidy (NT/T)-acute myeloid leukemia (AML) are rare and generally show poor survival. A 62-year-old man was referred to our hospital with pancytopenia. A bone marrow examination revealed the proliferation of extremely large blasts, and led to the diagnosis of AML M0. A cytogenetic analysis showed an NT-karyotype of 91, XXYY, -5, add(18)(p21),del(20)(q12q13)×2. Complete remission was achieved with single remission induction chemotherapy. Although consolidation chemotherapies were not available because of his critical condition, he remained in remission and survived for more than 40 months without cytopenia. However, repeated bone marrow examinations showed persistent clonal hematopoiesis with del(20)(q12q13) without apparent myelodysplasia.

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“…Two further cases of patients over 65 years old enlarge our knowledge of a good sensitivity of GM-AML to SICT. A 67-year old man with de novo Near-Tetraploid (NT) GM-AML M0 with WBC 52.9×10 9 /L reached CR and survived 51 months in CR after three 3+7 cycles [24] while most European cases with de novo NT-AML were EMD-AML with 33 % CR rate and maximal survival 31.5 months after SICT [30]. In 2017-8 we treated an 86-year old man diagnosed with GM-AML, normal karyotype with NPM1 mutation and FLT3-ITD, WBC 33.7×10 9 /L in 2011, who reached CR after two cycles of These two cases of patients over 65 years with GM-AML and all of our 6 octogenarian cases treated with SICT had WBC over 15×10 9 /L, the threshold over which treatment with Hypomethylating Agents (HMA) azacytidine or decitabine has not been recommended [31,32].…”

Section: Discussionmentioning

confidence: 99%

“…The French-American-British (FAB) classification [23] with standard cytochemical and immunophenotyping methods was used for establishing the diagnosis of AML [23,17]. Cytogenetic and I-FISH analyses were made from 24-hour unstimulated cultures of patients' bone marrow cells at diagnosis [17,24]. Molecular-genetic analyses were performed on cDNA obtained by reverse transcription of RNA (or for CEBPA from genomic DNA by sequencing the multiplied entire coding region of CEBPA) from bone marrow mononuclear cells at diagnosis as reported [24].…”

Section: Study Groupmentioning

confidence: 99%

“…Cytogenetic and I-FISH analyses were made from 24-hour unstimulated cultures of patients' bone marrow cells at diagnosis [17,24]. Molecular-genetic analyses were performed on cDNA obtained by reverse transcription of RNA (or for CEBPA from genomic DNA by sequencing the multiplied entire coding region of CEBPA) from bone marrow mononuclear cells at diagnosis as reported [24]. Morphological dysplastic features of erythroblasts, megakaryocytes, and granulocytes, in diagnostic Bone Marrow (BM) smears from patients were evaluated according to the FAB criteria [25] independently by two experienced hematologists (PL,JG) [17].…”

Section: Study Groupmentioning

confidence: 99%

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De Novo Acute Myeloid Leukemia Involving only Granulocyte-Macrophage Line in Octogenarians with Leucocytes over 15 × 109/L Exhibit a Favorable Response to Standard-Dose Induction Chemotherapy

Lemež¹

2018

Ann Hematol Oncol

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Objectives: Octogenarians with Acute Myeloid Leukemia (AML) exhibit poor median survival of 1-3 months after Standard-Dose Induction Chemotherapy (SICT). De novo AML without Erythroblastic And/Or Megakaryocytic Dysplasia (EMD) involving only Granulocyte-Macrophage Line (GM-AML) is associated with a 74-86% Complete Remission (CR) rate after SICT and improved survival in adult patients aged below 65 years. Our aim was to study if octogenarians with GM-AML will achieve CR and improved survival after SICT.Patients and Methods: Consecutive 12 octogenarians with de novo non-M3 AML with 48-94% (median 80%) bone marrow blasts, classified as AML FAB types: 2×M2, 7×M4, and 3×M5 were offered 3+7 type of SICT. Criteria for EMD were dyserythropoiesis 30% and/or dysmegakaryopoiesis 50% or higher.Results: Six cases, all with leucocytes over 15×10 9 /L, opted for SICT. Three cases with GM-AML achieved CR and survived 16.5-28 months on maintenance therapy while 3 cases with EMD-AML did not reach CR and had survival 0.4-2.7 months. Genetic findings in GM-AML cases showed 46, XY, del (12) (p13) with deleted ETV6 gene, 46, XX with NPM1 mutation and negative for FLT3-ITD and in the third case normal karyotype, which was found in two resistant EMD-AML cases. Survival 0.2-4.0 months was observed in the 6 remaining patients (two with GM-AML, 4 with EMD-AML) on palliative or supportive treatment. Conclusion:Octogenarians with GM-AML even with poor performance status (2×PS 3) may achieve CR and survival benefit after SICT, which is further supported by two other elderly GM-AML cases with over 5 years survival.

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Unusually Long Survival of a 67-Year-Old Patient with Near-Tetraploid Acute Myeloid Leukemia M0 without Erythroblastic and Megakaryocytic Dysplasia

Cited by 6 publications

References 48 publications

Tetraploidy acute myeloid leukaemia after chromosome 16 inversion

Tetraploidy acute myeloid leukaemia after chromosome 16 inversion

Near Tetraploidy Acute Myeloid Leukemia in Long-term Remission with Persistent Clonal Hematopoiesis with del(20)(q12q13)

De Novo Acute Myeloid Leukemia Involving only Granulocyte-Macrophage Line in Octogenarians with Leucocytes over 15 × 109/L Exhibit a Favorable Response to Standard-Dose Induction Chemotherapy

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