Because of its stimulating effect on RBC production, erythropoietin (Epo) is used to treat anemia, for example, in patients on dialysis or on chemotherapy. In <i  >&#x3b2;</i>-thalassemia, where Epo levels are low relative to the degree of anemia, Epo treatment improves the anemia state. Since RBC and platelets of these patients are under oxidative stress, which may be involved in anemia and thromboembolic complications, we investigated Epo as an antioxidant. Using flow-cytometry technology, we found that in vitro treatment with Epo of blood cells from these patients increased their glutathione content and reduced their reactive oxygen species, membrane lipid peroxides, and external phosphatidylserine. This resulted in reduced susceptibility of RBC to undergo hemolysis and phagocytosis. Injection of Epo into heterozygous (Hbb<i  ><sup>th3/+</sup></i>) <i  >&#x3b2;</i>-thalassemic mice reduced the oxidative markers within 3 hours. Our results suggest that, in addition to stimulating RBC and fetal hemoglobin production, Epo might alleviate symptoms of hemolytic anemias as an antioxidant.

Johnny Amer

Mutaz Dana

Eitan Fibach

The Antioxidant Effect of Erythropoietin on Thalassemic  Blood Cells

All ancient nations hinged their beliefs about hema (blood) on their religious dogmas as related to mythology or the origins of religion. The Hellenes (Greeks) especially have always known hema as the well-known red fluid of the human body. Greek scientific considerations about blood date from Homeric times. The ancient Greeks considered hema as synonymous with life. In Greek myths and historical works, one finds the first references to the uninterrupted vascular circulation of blood, the differences between venous and arterial blood, and the bone marrow as the site of blood production. The Greeks also speculated about mechanisms of blood coagulation and the use of blood transfusion to save life.

John Meletis

Kostas Konstantopoulos

The Beliefs, Myths, and Reality Surrounding the Word Hema (Blood) from Homer to the Present

Fanconi anemia (FA) is a recessively inherited disease characterized by multiple symptoms including growth retardation, skeletal abnormalities, and bone marrow failure. The FA diagnosis is complicated due to the fact that the clinical manifestations are both diverse and variable. A chromosomal breakage test using a DNA cross-linking agent, in which cells from an FA patient typically exhibit an extraordinarily sensitive response, has been considered the gold standard for the ultimate diagnosis of FA. In the majority of FA patients the test results are unambiguous, although in some cases the presence of hematopoietic mosaicism may complicate interpretation of the data. However, some diagnostic overlap with other syndromes has previously been noted in cases with Nijmegen breakage syndrome. Here we present results showing that misdiagnosis may also occur with patients suffering from two of the three currently known cohesinopathies, that is, Roberts syndrome (RBS) and Warsaw breakage syndrome (WABS). This complication may be avoided by scoring metaphase chromosomes—in addition to chromosomal breakage—for spontaneously occurring premature centromere division, which is characteristic for RBS and WABS, but not for FA.

Petra van der Lelij

Anneke B. Oostra

Martin A. Rooimans

Hans Joenje

Johan P. de Winter

Diagnostic Overlap between Fanconi Anemia and the Cohesinopathies: Roberts Syndrome and Warsaw Breakage Syndrome

Reticulocyte hemoglobin content (CHr) and percentage of hypochromic cells
(%Hypo) are restricted to the Siemens analysers. 
The aims of the study were to investigate the correlation of Red cells size factor
(RSf) and low Hemoglobin density (LHD%), reported by Beckman-Coulter
analysers, with CHr and %Hypo in the assessment of iron status in the presence of
inflammation. 
381 samples were run on both LH 750 (Beckman-Coulter) and Advia 2120 (Siemens)
analysers. Correlation between parameters were calculated and the diagnostic
performance of the new parameters was assessed. 
<i  >Results</i>. Correlation between RSf and CHr, <svg style="vertical-align:-0.17555pt;width:53.237499px;" id="M1" height="11.1" version="1.1" viewBox="0 0 53.237499 11.1" width="53.237499"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,11.1)">
		<g transform="translate(72,-63.12)">
			<text transform="matrix(1,0,0,-1,-71.95,63.35)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑟</tspan>
				<tspan style="font-size: 12.50px; " x="8.5645552" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="20.604944" y="0">0</tspan>
				<tspan style="font-size: 12.50px; " x="26.856443" y="0">.</tspan>
				<tspan style="font-size: 12.50px; " x="29.982195" y="0">8</tspan>
				<tspan style="font-size: 12.50px; " x="36.233692" y="0">5</tspan>
			</text>

		</g>
	</g>
</svg>. ROC curve analysis for RSf in the diagnosis of iron restricted erythropoiesis defined
as CH<svg style="vertical-align:-0.30096pt;width:40.349998px;" id="M2" height="11.0625" version="1.1" viewBox="0 0 40.349998 11.0625" width="40.349998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,11.0625)">
		<g transform="translate(72,-63.15)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">r</tspan>
				<tspan style="font-size: 12.50px; " x="7.6268301" y="0"><</tspan>
				<tspan style="font-size: 12.50px; " x="19.667219" y="0">2</tspan>
				<tspan style="font-size: 12.50px; " x="25.918718" y="0">8</tspan>
			</text>

		</g>
	</g>
</svg> pg: AUC 0.983; Cutoff 91.1%; Sensitivity 98.8%; Specificity 89.6% Correlation between LHD% and %Hypo, <svg style="vertical-align:-0.27588pt;width:61.049999px;" id="M3" height="11.1625" version="1.1" viewBox="0 0 61.049999 11.1625" width="61.049999"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,11.1625)">
		<g transform="translate(72,-63.07)">
			<text transform="matrix(1,0,0,-1,-71.95,63.4)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑟</tspan>
				<tspan style="font-size: 12.50px; " x="8.5645552" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="20.604944" y="0">0</tspan>
				<tspan style="font-size: 12.50px; " x="26.856443" y="0">.</tspan>
				<tspan style="font-size: 12.50px; " x="29.982195" y="0">8</tspan>
				<tspan style="font-size: 12.50px; " x="36.233692" y="0">6</tspan>
				<tspan style="font-size: 12.50px; " x="42.485195" y="0">9</tspan>
			</text>

		</g>
	</g>
</svg>. 
ROC curve analysis for LHD% in the diagnosis of iron deficiency defined by
%Hypo <svg style="vertical-align:-0.30096pt;width:22.987499px;" id="M4" height="11.25" version="1.1" viewBox="0 0 22.987499 11.25" width="22.987499"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,11.25)">
		<g transform="translate(72,-63)">
			<text transform="matrix(1,0,0,-1,-71.95,63.35)">
				<tspan style="font-size: 12.50px; " x="0" y="0">></tspan>
				<tspan style="font-size: 12.50px; " x="12.027886" y="0">5</tspan>
			</text>

		</g>
	</g>
</svg>%: AUC 0.954; Cut off 6.0%; Sensitivity 96.6%; Specificity 83.2%
<i  >Conclusions</i>. RSf and LHD% could be reliable parameters for the study of iron
metabolism status.

Eloísa Urrechaga

Luís Borque

Jesús F. Escanero

Erythrocyte and Reticulocyte Indices on the LH 750 as Potential Markers of Functional Iron Deficiency

The main cause of morbidity and mortality in Fanconi anemia patients is the development of bone marrow (BM) failure; thus correction of hematopoietic stem cells (HSCs) through gene transfer approaches would benefit FA patients. However, gene therapy trials for FA patients using ex vivo transduction protocols have failed to provide long-term correction. In addition, ex vivo cultures have been found to be hazardous for FA cells. To circumvent negative effects of ex vivo culture in FA stem cells, we tested the corrective ability of direct injection of recombinant lentiviral particles encoding FancC-EGFP into femurs of <svg     style="vertical-align:-0.23206pt;width:66.212502px;" id="M1" height="16.9375" version="1.1" viewBox="0 0 66.212502 16.9375" width="66.212502"  xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg">
    
        
            <g transform="matrix(.017,-0,0,-.017,.062,16.588)"><path id="x1D439" d="M599 650l-18 -160l-30 -2q-2 70 -11 94q-5 16 -26 24t-75 8h-94q-27 0 -33.5 -6.5t-12.5 -32.5l-43 -226h108q52 0 72.5 5t31 18t28.5 52h28l-40 -200h-28q-1 42 -6 56.5t-24.5 21t-70.5 6.5h-108l-32 -177q-13 -65 1 -80.5t88 -22.5l-8 -28h-279l6 28q61 5 77.5 20.5&#xA;t29.5 81.5l72 387l7.5 40.5t1 26.5t-4 18t-15.5 10t-24 6t-37 4l8 28h461z" /></g><g transform="matrix(.017,-0,0,-.017,10.636,16.588)"><path id="x1D44E" d="M483 97q-42 -50 -88.5 -79.5t-68.5 -29.5q-37 0 -17 93l22 102h-2q-54 -79 -144 -149q-59 -46 -100 -46q-24 0 -43 29t-19 86q0 78 34.5 153t94.5 120q41 31 94 51.5t98 20.5q29 0 72 -9q26 -6 39 -6l2 -4q-30 -117 -67 -323q-8 -41 2 -41q16 0 79 58zM374 387&#xA;q-32 15 -73 15q-52 0 -83 -23q-48 -36 -78 -108.5t-30 -152.5q0 -33 8.5 -50.5t20.5 -17.5q31 0 107 79t99 132q15 40 29 126z" /></g><g transform="matrix(.017,-0,0,-.017,19.237,16.588)"><path id="x1D45B" d="M495 86q-46 -47 -87 -72.5t-63 -25.5q-43 0 -16 107l49 210q7 34 8 50.5t-3 21t-13 4.5q-35 0 -109.5 -72.5t-115.5 -140.5q-21 -75 -38 -159q-50 -10 -76 -21l-6 8l84 340q8 35 -4 35q-17 0 -67 -46l-15 26q44 44 85.5 70.5t64.5 26.5q35 0 10 -103l-24 -98h2&#xA;q42 56 97 103.5t96 71.5q46 26 74 26q9 0 16 -2.5t14 -11.5t9.5 -24.5t-1 -44t-13.5 -68.5q-30 -117 -47 -200q-4 -19 -3.5 -25t6.5 -6q21 0 70 48z" /></g><g transform="matrix(.017,-0,0,-.017,27.77,16.588)"><path id="x1D450" d="M383 397q0 -32 -35 -49q-12 -6 -23 8q-37 45 -84 45t-90 -71q-40 -65 -40 -167q0 -57 22 -86t59 -29q38 0 81.5 24.5t69.5 51.5l16 -21q-44 -53 -104 -84t-109 -31q-56 0 -89.5 41t-33.5 117q0 61 30 124t79 105q33 28 81 50.5t86 22.5q34 0 59 -15.5t25 -35.5z" /></g><g transform="matrix(.017,-0,0,-.017,34.672,16.588)"><path id="x1D436" d="M682 629q-1 -16 -1.5 -72t-2.5 -86l-31 -4q-5 92 -51 129t-139 37q-100 0 -177 -49t-116 -125t-39 -162q0 -122 66 -201t182 -79q83 0 137 42.5t112 128.5l26 -15q-12 -31 -42.5 -88t-45.5 -75q-139 -27 -199 -27q-148 0 -243 81.5t-95 226.5q0 173 129.5 274.5&#xA;t325.5 101.5q114 0 204 -38z" /></g>

            <g transform="matrix(.012,-0,0,-.012,46.65,8.425)"><path id="x2212" d="M535 230h-483v50h483v-50z" /></g><g transform="matrix(.012,-0,0,-.012,53.635,8.425)"><path id="x2F" d="M368 703l-264 -866h-60l265 866h59z" /></g><g transform="matrix(.012,-0,0,-.012,58.537,8.425)"><use xlink:href="#x2212"/></g>

        
    
</svg> mice. Using this approach, we show that <svg     style="vertical-align:-0.23206pt;width:66.212502px;" id="M2" height="16.9375" version="1.1" viewBox="0 0 66.212502 16.9375" width="66.212502"  xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg">
    
        
            <g transform="matrix(.017,-0,0,-.017,.062,16.588)"><use xlink:href="#x1D439"/></g><g transform="matrix(.017,-0,0,-.017,10.636,16.588)"><use xlink:href="#x1D44E"/></g><g transform="matrix(.017,-0,0,-.017,19.237,16.588)"><use xlink:href="#x1D45B"/></g><g transform="matrix(.017,-0,0,-.017,27.77,16.588)"><use xlink:href="#x1D450"/></g><g transform="matrix(.017,-0,0,-.017,34.672,16.588)"><use xlink:href="#x1D436"/></g>

            <g transform="matrix(.012,-0,0,-.012,46.65,8.425)"><use xlink:href="#x2212"/></g><g transform="matrix(.012,-0,0,-.012,53.635,8.425)"><use xlink:href="#x2F"/></g><g transform="matrix(.012,-0,0,-.012,58.537,8.425)"><use xlink:href="#x2212"/></g>

        
    
</svg> HSCs were efficiently corrected. Intrafemoral gene transfer of the <svg     style="vertical-align:-0.23206pt;width:46.712502px;" id="M3" height="11.75" version="1.1" viewBox="0 0 46.712502 11.75" width="46.712502"  xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg">
    
        
            <g transform="matrix(.017,-0,0,-.017,.062,11.4)"><use xlink:href="#x1D439"/></g><g transform="matrix(.017,-0,0,-.017,10.636,11.4)"><use xlink:href="#x1D44E"/></g><g transform="matrix(.017,-0,0,-.017,19.237,11.4)"><use xlink:href="#x1D45B"/></g><g transform="matrix(.017,-0,0,-.017,27.77,11.4)"><use xlink:href="#x1D450"/></g><g transform="matrix(.017,-0,0,-.017,34.672,11.4)"><use xlink:href="#x1D436"/></g>

        
    
</svg> gene prevented the mitomycin C-induced BM failure. Moreover, we show that intrafemoral gene delivery into aplastic marrow restored the bone marrow cellularity and corrected the remaining HSCs. These results provide evidence that targeting FA-deficient HSCs directly in their environment enables efficient and long-term correction of BM defects in FA.

Ouassila Habi

Johanne Girard

Valérie Bourdages

Marie-Chantal Delisle

Madeleine Carreau

Correction of Fanconi Anemia Group C Hematopoietic Stem Cells Following Intrafemoral Gene Transfer

About this journal

<p><em>Anemia</em> publishes original research articles and review articles on all types of anemia. Articles focus on patient care, health systems, and epidemiology, among other relevant topics.</p>


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Anemia publishes original research articles and review articles on all types of anemia. Articles focus on patient care, health systems, and epidemiology, among other relevant topics.

Anemia