Eosinophilic fasciitis associated with hypereosinophilia, abnormal bone-marrow karyotype and inversion of chromosome 5

2013 ◽  
Vol 39 (2) ◽  
pp. 150-153 ◽  
Author(s):  
J. S. Ferguson ◽  
J. Bosworth ◽  
T. Min ◽  
J. Mercieca ◽  
C. A. Holden
Keyword(s):  
Bone Marrow ◽  
Eosinophilic Fasciitis ◽  
Chromosome 5 ◽  
Abnormal Bone Marrow ◽  
And Inversion

Chronic Myelomonocytic Leukemia With Abnormal Bone Marrow Eosinophils

2003 ◽  
Vol 127 (9) ◽  
pp. 1214-1216
Author(s):  
Jason Hyde ◽  
Tsieh Sun
Keyword(s):  
Bone Marrow ◽  
Eosinophil Count ◽  
Chronic Myelomonocytic Leukemia ◽  
Small Subset ◽  
Rare Entity ◽  
Abnormal Bone Marrow ◽  
Acute Myelomonocytic Leukemia ◽  
Peripheral Eosinophil ◽  
Myelomonocytic Leukemia

Abstract Chronic myelomonocytic leukemia with eosinophilia is a recently defined rare entity frequently associated with t(5;12)(q33;p13) translocation. It usually shows a peripheral eosinophil count greater than 1500/μL. However, the literature contains a small subset of cases in which the major manifestation is bone marrow eosinophilia. We report a case similar to that subset and discuss our finding that the immature eosinophils are identical to those seen in acute myelomonocytic leukemia with abnormal bone marrow eosinophils.

Tissue Engineering of Normal and Abnormal Bone Marrow☆

2016 ◽  
Author(s):  
T. Mortera-Blanco ◽  
M. Rende ◽  
N. Panoskaltsis ◽  
A. Mantalaris
Keyword(s):  
Tissue Engineering ◽  
Bone Marrow ◽  
Abnormal Bone Marrow

The Abnormal Bone Marrow: MRI Patterns

2014 ◽  
pp. 35-56
Author(s):  
Lia Angela Moulopoulos ◽  
Vassilis Koutoulidis
Keyword(s):  
Bone Marrow ◽  
Abnormal Bone Marrow

Slit2 Increases Hematopoietic Stem Cell Numbers in Mice

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 77-77
Author(s):  
Amanda J Waterstrat ◽  
Ying Liang ◽  
Hartmut Geiger ◽  
Gary Van Zant
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Stem Cell ◽  
Confidence Interval ◽  
Ectopic Expression ◽  
Pcr Analysis ◽  
Chromosome 5 ◽  
Hematopoietic Stem ◽  
Congenic Mice ◽  
Background Strain

Abstract A complex interaction of cell-intrinsic and extra-cellular signals cooperate to determine the number and behavior of Hematopoietic Stem Cells (HSCs). Elucidation of these regulatory networks promises to offer novel insights into HSC biology and HSC-mediated clinical therapies. In an effort to identify cell-intrinsic, genetic regulatory mechanisms determining HSC number, we initiated a forward genetic analysis beginning with HSC quantification in inbred mice using the Cobblestone Area Forming Cell (CAFC) assay. Subsequent linkage analysis revealed that the 3–7 fold larger HSC population in young DBA/2 relative to C57BL/6 mice was linked to multiple quantitative trait loci (QTL), including a locus with peak linkage (LOD = 3.1) at the 40 Mb position on chromosome 5 with a 95% confidence interval ranging from 29.3–55 Mbp. Congenic strains were generated on both the C57BL/6 and DBA/2 backgrounds in which the chromosome 5 QTL was exchanged between the strains by selective, genotype-assisted breeding. The DBA/2 interval increased HSC number 2.4 fold relative to the C57BL/6 background strain while the C57BL/6 QTL decreased HSC number 2 fold relative to the DBA/2 strain. Gene expression profiling of Lineage negative, Sca-1+, c-Kit+ (LSK) cells from C57BL/6, DBA/2 and congenic mice revealed 6 differentially expressed candidate genes in the 95% confidence interval among the 46,644 probes on the array. Among them a single transcript, Slit2, was expressed in a pattern correlated with stem cell number in both congenic-background strain comparisons and could be verified by RT-PCR analysis. Slit2 expression was positively correlated with HSC number and highly enriched in LSK cells of inbred and congenic mice bearing the DBA/2 genotype at the chromosome 5 QTL. A retrovirus was used to stably infect HSC-enriched C57BL/6 bone marrow cells, which normally do not express Slit2, with a Slit2-containing GFP vector, resulting in ectopic expression of the Slit2 transcript in GFP+ cells. Infected cells were then transplanted into sub-lethally irradiated C57BL/6 hosts and expanded in vivo for 12 weeks ensuring reconstitution of the complete hematopoietic hierarchy within the GFP+ fraction. CAFC analysis of GFP+ cells revealed that the ectopic expression of Slit2 resulted in a 2-fold increase in HPC/HSC numbers relative to an empty vector control. On the basis of this finding we demonstrate for the first time that expression of Slit2 by HSCs results in expansion of the HSC population. Slit/Roundabout (Robo) signaling is required in embryonic and neuronal development and has recently been shown to play important roles in the migration and function of a growing list of non-neuronal cells including a variety of cancer cell types. Future studies will aim to determine if Slit2 expression influences the interaction between HSCs and the microenvironment in a manner that promotes expansion of the HSC compartment, perhaps by overriding quiescence cues from the niche and/or altering the spatial orientation of stem cells within the bone marrow microenvironment.

Increases In Mirna-145 and Mirna-146a Expression In Patients with IPSS Lower-Risk Myelodysplastic Syndromes and Del(5q) Treated with Lenalidomide.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3631-3631 ◽  
Author(s):  
Esther Natalie Oliva ◽  
Francesco Nobile ◽  
Pasquale Iacopino ◽  
Giuliana Alimena ◽  
Francesco Di Raimondo ◽  
...  
Keyword(s):  
Gene Expression ◽  
Bone Marrow ◽  
Messenger Rna ◽  
Lower Risk ◽  
Healthy Subjects ◽  
Detection System ◽  
Nature Medicine ◽  
Chromosome 5 ◽  
Hematopoietic Stem ◽  
Preliminary Results

Abstract Abstract 3631 Introduction: The erythroid differentiation defect observed in 5q– syndrome has been attributed to the RPS14 gene located within the CDR of the long arm of chromosome 5. We have recently demonstrated that RPS14 expression increases during lenalidomide treatment. However, haploinsufficiency of RPS14, which encodes ribosomal protein S14, does not explain clonal dominance. The expression of miRNAs, miR-145 (5q33.1) and miR-146a (5q33.3), in CD34+ bone marrow (BM) cells of individuals with MDS with deletion of the long arm of chromosome 5 (del(5q)) is lower compared to normal controls (Starczynowski et al, Nature Medicine, 2010). miRNAs are small noncoding RNAs that post-transcriptionally repress specific messenger RNA targets through interaction with the 3′ untranslated region (UTR). Loss of noncoding transcripts encoding miRNAs within the CDR may result in haploinsufficiency by loss of inhibition of their targets. Concurrent loss of both miR-145 and miR-146a resulted in activation of innate immune signalling through elevated expression of their respective targets, TIRAP and TRAF6. Furthermore, knockdown of miR-145 and miR-146a or overexpression of TRAF6 in mouse HSPC (Hematopoietic stem and progenitor cells) recapitulated features of 5q– syndrome, such as bone marrow dysplasia, anemia and thrombocytosis. We present preliminary results of changes in miRNA expression in IPSS lower-risk MDS with del(5q) during treatment with lenalidomide. Methods: A prospective single-arm trial investigating the efficacy and safety of lenalidomide in 46 patients with MDS with del(5q) with/without additional cytogenetic abnormalities and Hb < 10 g/dL. Lenalidomide was administered orally at a starting dose of 10 mg/day for a maximum of 12 months. When necessary, dosing was reduced to 5 mg/day or 5 mg on alternate days. Bone marrow assessments were performed at baseline and every 3 months, thereafter. For the evaluation of miRNA-145 and miRNA-146a in patient samples, 300 ng/μl of miRNAs were isolated in each purified BM sample by using mirVana™ miRNA Isolation Kit-Ambion and TaqMan miRNA Array Analysis was performed to determine the expression of miRNAs (7900HT Sequence Detection System Applied Biosystems). Patient BM-miRNAs were calibrated with miRNAs from BM of healthy volunteer donors. It was assumed that BM expression value of each calibrator miRNA was 1 unit. RPS14 gene assays were performed using TaqMan® Low Density Array Fluidic card (TaqMan® Human Array, Applied Biosystems, Foster City, CA, USA) based on Applied Biosystems PRISM® 7900HT comparative ddCT method, according to the manufacturer's instructions. Target gene expression levels were measured in triplicate and normalized against the expression of the 18S housekeeping gene from a BM pool of normal, healthy subjects at all timepoints. Median relative gene expression values in MDS patients were compared to healthy subjects, set as a value of 1. Results: Four patients have been evaluated (1 M, 3 F; ages 65, 66, 73 and 76 years, respectively) at baseline and after 12 weeks. At baseline, 2 patients were RBC-transfusion dependent. One patient had one additional cytogenetic abnormality (+8 in 15% metaphases). All patients obtained an erythroid response by week 12: mean Hb values significantly increased from 8.4 ± 0.9 at baseline to 11.6 ± 0.9 g/dL (p=0.01). All patients obtained a cytogenetic response, 2 of which were complete. miRNA-145 and miRNA-146a expression were both low at baseline and significantly increased by week 12 (Table). Conclusions: Preliminary results confirm that, in IPSS lower-risk MDS with del(5q), miRNA-145 and miRNA-146a expression is low. Lenalidomide treatment is associated with erythroid responses and cytogenetic remissions concurrent with significant increases in miRNA-145 and miRNA-146a expression. Disclosures: Oliva: Celgene: Consultancy.

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