Activated peripheral blood and endothelial cells in thalassemia patients.
OBJECTIVE
Thalassemia patients have alterations in the expression of some activation and adhesion molecules on peripheral blood lymphocytes. We studied cell surface antigens on peripheral blood cells associated with the activation of these cells and soluble molecules produced by activated endothelium.


METHODS
We investigated the expression of CD11b, CD18, CD35, CD43, CD44, and CD69 on the peripheral blood monocytes, Cd11b, CD18, CD35, CD43, CD44, CD67 on peripheral blood neutrophils and CD38 and CD69 on peripheral blood lymphocytes. We studied 68 transfusion-dependent thalassemics (group A), 10 transfusion non-dependent thalassemics (group B), 18 beta-thalassemia carriers (group C), and 28 normal individuals. Relative fluorescence intensity was used to determine the antigen density. Analysis was performed with an EPICS ELITE flow cytometer. Furthermore, soluble intercelullar adhesion molecule 1 (sICAM-1), soluble vascular adhesion molecule 1 (sVCAM-1), and E-selectin, tumor necrosis factor (TNF) alpha, and interleukin (IL) 1beta were measured in the plasma of patients by enzyme-linked immunometric assay.


RESULTS
The expression of CD11b, CD18, and CD69 on the monocytes of group A was significantly greater than in groups B and C and in controls, while CD44 was significantly downregulated in group A. CD11b, CD18, CD35, CD44, and CD67 on the surface of neutrophils and CD38 and CD69 on the surface of lymphocytes were also overexpressed in group A. CD44 was downregulated on the monocytes and upregulated on the neutrophils of the patients compared to controls. The levels of sICAM-1, sVCAM-1, E-selectin, TNF-alpha, and IL-1beta in the serum of patients in groups A and B were higher than those in group C and the controls.


CONCLUSIONS
Endothelial activation markers are significantly increased in thalassemia patients, and activated blood cells circulate in the peripheral blood. These may be related to the vascular complications in these patients and might be useful markers for the follow-up of the vascular disease.
Kyriakou
D S
DS
Department of Hematology, University Hospital of Heraklion, Crete, Greece. deskyriakou@hotmail.com
Alexandrakis
M G
MG
Kyriakou
E S
ES
Liapi
D
D
Kourelis
T V
TV
Passam
F
F
Papadakis
A
A
D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D018845:CD11 Antigens / Q000032:analysis; D018821:CD18 Antigens / Q000032:analysis; D002648:Child; D019040:E-Selectin / Q000097:blood; D004730:Endothelium, Vascular / Q000276:immunology*; D005260:Female; D006579:Heterozygote; D006801:Humans; D018960:Hyaluronan Receptors / Q000032:analysis; D016130:Immunophenotyping; D018799:Intercellular Adhesion Molecule-1 / Q000097:blood; D007375:Interleukin-1 / Q000097:blood; D037181:Lectins, C-Type; D051917:Leukosialin; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000502:physiology*; D017463:Receptors, Complement 3b / Q000032:analysis; D012795:Sialoglycoproteins / Q000032:analysis; D013789:Thalassemia / Q000097:blood* / Q000276:immunology; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis; D019010:Vascular Cell Adhesion Molecule-1 / Q000097:blood; D017086:beta-Thalassemia / Q000276:immunology; D000099192:CD69 Antigens
D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D018845:CD11 Antigens; D018821:CD18 Antigens; D019040:E-Selectin; D018960:Hyaluronan Receptors; D018799:Intercellular Adhesion Molecule-1; D007375:Interleukin-1; D037181:Lectins, C-Type; D051917:Leukosialin; D017463:Receptors, Complement 3b; D012795:Sialoglycoproteins; D014409:Tumor Necrosis Factor-alpha; D019010:Vascular Cell Adhesion Molecule-1; D000099192:CD69 Antigens; C465014:SPN protein, human