 | COOLEY'S ANEMIA: SEVENTH SYMPOSIUM
Copyright © 1998 by the New York Academy of Sciences
description
Annals of the New York Academy of Sciences 850:120-128 (1998)
© 1998 New York Academy of Sciences
Hydroxyurea Therapy in Thalassemiaa
DIMITRIS LOUKOPOULOSb,g,
ERSI VOSKARIDOUc,
ALEXANDRA STAMOULAKATOUd,
YANNIS PAPASSOTIRIOUd,
VASILIKI KALOTYCHOUb,
APHRODITE LOUTRADIc,
GABRIEL COZMAe,
HELENI TSIARTAf AND
NIKOS PAVLIDESf
bFirst Department of Medicine, University of Athens
cThalassemia Center, Laikon Hospital, Athens
dHematology Laboratory, Aghia Sophia Children's Hospital, Athens
eResearch Division, CILAG Company, Zug, Switzerland
fThalassemia Center, Nikosia Hospital, Cyprus
aThese studies were supported by grants 91 ED 97 (Secretariat of R and D) to D.L. and Y3A/3440 (Ministry of Health) to E.V.
gCorresponding author: Dimitris Loukopoulos, MD, Professor of Medicine, First Department of Medicine, University of Athens, Laikon Hospital, 115 27 Athens, Greece; Tel: 30-1-777 11 61; Tel. 30-1-363 33 79 (home); Fax: 301-360 7089; E-mail: dloukop{at}atlas.uoa.gr
The clinical effectiveness of Hydroxyurea in thalassemia is still controversial. The present paper puts together the authors' experience in two groups of patients with thalassemia intermedia and sickle cell/ß-thalassemia treated with varying dosages of hydroxyurea over several months. A third group received hydroxyurea along with recombinant human erythropoietin. Our observations are summarized in that treatment with hydroxyrea results in a significant increase of fetal hemoglobin with no change of the total hemoglobin levels. The drug causes also a considerable increase of the erythrocyte volume and hemoglobin content while the MCHC values remain unchanged. As a rule, and without objective criteria so far, patients state feeling better and having more energy. The authors postulate that this feeling may reflect the significant decrease of ineffective erythropoiesis resulting by the replacement of the poorly hemoglobinized, prematurely dying erythroid progenitor and red cell population by another population of cells with higher hemoglobin content and longer survival, the regeneration of which requires less energy and consumption. As expected, patients with sickle cell/ß-thalassemia have also fewer crises and painful episodes. The above findings are in keeping with the few available reports in the literature.
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