scholarly journals Acute myeloid leukemia with RAM immunophenotype: a pediatric case with unusual morphologic features

2017 ◽  
Vol 9 (2) ◽  
Author(s):  
Miriam Conces ◽  
Rolla Abu-Arja ◽  
Suzanne Reed ◽  
Hemalatha G. Rangarajan ◽  
Terri L. Guinipero ◽  
...  
Keyword(s):  
Myeloid Leukemia ◽  
Poor Prognosis ◽  
Myelogenous Leukemia ◽  
Cell Transplant ◽  
Hematopoietic Cell Transplant ◽  
Pediatric Case ◽  
Acute Myelogenous ◽  
Distinct Subtype ◽  
Acute Myeloid

The RAM immunophenotype has been recently described as a subtype of acute myelogenous leukemia (AML) that is characterized clinically by extremely poor prognosis. We present a case of AML with RAM immunophenotype in a 5-year-old patient that resulted in poor outcome despite early hematopoietic cell transplant. We describe the unusual morphologic features that, along with the distinct immunophenotype, may provide initial diagnostic clues and further justify the classification of this AML variant as a rather distinct subtype.

Loss of Structural Maintenance of Chromosomes 1A Protein Expression Is Associated with a Poor Prognosis in Acute Myelogenous Leukemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4693-4693
Author(s):  
Utz O. Krug ◽  
Claudia Hömme ◽  
Nicola Tidow ◽  
Horst Buerger ◽  
Gabriele Koehler ◽  
...  
Keyword(s):  
Protein Expression ◽  
Myeloid Leukemia ◽  
Poor Prognosis ◽  
Myelogenous Leukemia ◽  
Newly Diagnosed ◽  
Event Free Survival ◽  
Free Survival ◽  
Acute Myelogenous ◽  
Structural Maintenance Of Chromosomes ◽  
Acute Myeloid

Abstract Abstract 4693 Loss of Structural Maintenance of Chromosomes 1A Protein Expression is Associated with a Poor Prognosis in Acute Myeloid Leukemia Utz Krug, Claudia Hömme, Nicola Tidow, Horst Bürger, Gabriele Köhler, Achim Heinecke, Thomas Büchner, Wolfgang E. Berdel, Steffen Koschmieder, Carsten Müller-Tidow Introduction Acute myelogenous leukemia is a genetically heterogenous disease with many risk factors for a poor prognosis. One of the most important independent risk factor in AML is the age at diagnosis. Older patients with AML generally have a poor prognosis which suggests that the biology of AML in elderly patients differs from AML in younger patients. Methods Gene expression profiling was carried out to identify age related changes in AML blasts of 67 AML patients of different age (range: 17 to 80 years). Among the genes that correlated with age, SMC1A was selected for protein expression studies. A tissue array was created containing bone marrow histology samples of 135 patients with newly diagnosed AML of different ages and probed with an antibody against SMC1A and protein expression was quantified by the DAKO score. Results 131 genes showed a significant correlation between mRNA expression levels and patient age. Increasing age was associated with significantly decreased mRNA levels of SMC1A. 116 patient samples were evaluable for SMC1A protein expression and expression of SMC1A protein was low or absent in 74 out of 116 AML specimens. SMC1A protein expression did not show a correlation with patients' age at diagnosis. Both event free survival (2.6 months vs. 10.3 months, p=0.003, see figure) and overall survival (10.4 months vs. 22.6 months, p=0.015, see figure) were significantly worse in patients with low or absent SMC1A protein expression. In a multivariate analysis, SMC1A protein expression level remained a significant prognostic factor for event free survival (p=0.014) with a borderline significance for overall survival (p=0.066). Conclusions We identified 131 genes with putative age-dependent microarray mRNA expression and identified low levels of SMC1A protein expression as a marker for poor prognosis in patients with newly diagnosed acute myeloid leukemia. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Hematopoietic cell transplant for acute myeloid leukemia and myelodysplastic syndrome: conditioning regimen intensity

2018 ◽  
Vol 2 (16) ◽  
pp. 2095-2103 ◽  
Author(s):  
Mary Eapen ◽  
Ruta Brazauskas ◽  
Michael Hemmer ◽  
Waleska S. Perez ◽  
Patricia Steinert ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myelodysplastic Syndrome ◽  
Clinical Remission ◽  
Myeloid Leukemia ◽  
Conditioning Regimen ◽  
Hematopoietic Cell ◽  
Cell Transplant ◽  
Hematopoietic Cell Transplant ◽  
Key Points ◽  
Acute Myeloid

Key Points Bu4/Cy, Flu/Bu4, and Flu/Mel are optimal regimens for patients with AML in clinical remission or those with MDS. Flu/Mel, considered a less-intense regimen, is ideal for less fit patients.

scholarly journals Translocations and deletions of 5q13.1 in myelodysplasia and acute myelogenous leukemia: evidence for a novel critical locus

Blood ◽  
1996 ◽  
Vol 88 (6) ◽  
pp. 2259-2266 ◽  
Author(s):  
J Fairman ◽  
RY Wang ◽  
H Liang ◽  
L Zhao ◽  
D Saltman ◽  
...  
Keyword(s):  
Myeloid Leukemia ◽  
Interstitial Deletion ◽  
Negative Regulator ◽  
Myelogenous Leukemia ◽  
Chromosome 5 ◽  
Entire Chromosome ◽  
Acute Myelogenous ◽  
Paracentric Inversions ◽  
Acute Myeloid

Acquired partial and complete deletions of chromosome 5 (5q-, -5) are common cytogenetic anomalies associated with myelodysplasia (MDS) and acute myeloid leukemia (AML). A critical region of consistent loss at 5q31.1 (in > 90% of cases) has led us and others to postulate the presence of a key negative regulator(s) of leukemogenesis. Although the interstitial deletion limits vary among patients, del(5) (q13q33) and del(5)(q13q35) constitute major subsets. Furthermore, it is not rare to encounter deletions, translocations, or paracentric inversions involving 5q11 to 5q13, which indicates inactivation or disruption of important gene(s) at that locus. In this report, we have localized a novel locus at 5q13.1 to a 2.0-Mb interval between the anonymous markers D5S672 and GATA-P1804. This locus resided within the region of loss in 12 of 27 patients with anomalies of chromosome 5; one of these cases had apparent retention of both alleles of all the telomeric loci. Fluorescence in situ hybridization (FISH) studies demonstrate that the AML cell line ML3 is disrupted at 5q13.1 by a translocation involving chromosome 3, with apparent retention of the entire chromosome 5 sequence. Our results suggest that this novel proximal locus encodes a critical gene that may be deleted or disrupted in a subset of MDS/AML patients with chromosome 5 anomalies.

Results of High Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with Acute Myeloid Leukemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5438-5438
Author(s):  
Kenneth A. Ault ◽  
Delvyn Caedren Case ◽  
Marjorie A. Boyd ◽  
Thomas J. Ervin ◽  
Frederick Aronson ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Acute Myelogenous Leukemia ◽  
Myeloid Leukemia ◽  
Medical Center ◽  
Myelogenous Leukemia ◽  
High Dose ◽  
Transplant Program ◽  
Maine Medical ◽  
Acute Myelogenous ◽  
Acute Myeloid

Abstract 43 patients with acute myeloid leukemia have undergone transplantation at our institution over the past 14 years. Patient selection criteria included age less than 70 years, creatinine less than 2mg/ml, no active infection, cardiac ejection fraction >40%, DLCO > 50% of predicted and no other co-morbid conditions that would jeopardize survival. 39 patients were in first remission, 4 were in second or higher remission. 3 patients had favorable cytogenetics, 40 had intermediate or unfavorable cytogenetics. After achieving remission for at least 30 days, patients were consolidated with Etoposide and AraC, followed by G-CSF. Hematopoietic stem cells were collected when the WBC rebounded to at least 10,000/μl. The target dose of CD34 positive cells was 5×106/kg. The minimum dose given was 2.3 × 106/kg). High dose therapy consisted of Busulfan 1mg/kg and Etoposide 60mg/kg. The average age at transplantation was 42 years (range 20 to 61). Days of neutropenia (AGC<500/μl) ranged from 2 to 10 (average 5.2). The median length of follow up is 4.0 years. Kaplan-Meier progression-free survival is 38% at 5 years and 35% at 10 years. Currently 26 patients are alive, and 23 are free from progression. Overall survival is 60%. Maine Medical Center Autologous HPC Transplant Program Acute Myelogenous Leukemia Maine Medical Center Autologous HPC Transplant Program Acute Myelogenous Leukemia

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