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Substitutive-Inhibitory Therapy in Constitutional Non-Spherocytic Erythrocytic Disease

Substitutive-Inhibitory Therapy in Constitutional Non-Spherocytic Erythrocytic Disease Acta haem al..?/: 303-309 (19b4). Fundación Hematológica M ar del Plata, Argentina Substitutive-Inhibitory Therapy in Constitutional Non-Spherocytic Erythrocytic Disease By E n r i q u e R e w a l d Although large tracks of population have not been investigated, there is no doubt about the extensive distribution of hemolytic diseases, specially due to the high incidence of abnormal hemo­ globins in M editerranean countries and in certain areas of Asia and Africa. The severity of the homozygous state induced inter­ esting speculations about the disappearance of some isolated communities (1). The difference between hypo- and hyper-regenerative anem ia is fundamental. W ith identical low Hb. concentration a patient is able to live norm ally (simple aplastic disease) or his activities are limited by easy fatigability (hyperactive erythropoiesis) (fig. 1). In adults, bone marrow volume is about 2.600 cc. (2), equal to 5/9 of blood volume. Normally, h alf of it is in activity with 6% of nucleated cells (3). Fat marrow can be replaced in a few days (4) due to the regenerative potential of hemopoiesis. C r o s b y a n d A k e r o y d (5) calculated that as http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

Substitutive-Inhibitory Therapy in Constitutional Non-Spherocytic Erythrocytic Disease

Acta Haematologica , Volume 31 (5): 7 – Jan 1, 2009

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Publisher
Karger
Copyright
© 1964 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000209641
Publisher site
See Article on Publisher Site

Abstract

Acta haem al..?/: 303-309 (19b4). Fundación Hematológica M ar del Plata, Argentina Substitutive-Inhibitory Therapy in Constitutional Non-Spherocytic Erythrocytic Disease By E n r i q u e R e w a l d Although large tracks of population have not been investigated, there is no doubt about the extensive distribution of hemolytic diseases, specially due to the high incidence of abnormal hemo­ globins in M editerranean countries and in certain areas of Asia and Africa. The severity of the homozygous state induced inter­ esting speculations about the disappearance of some isolated communities (1). The difference between hypo- and hyper-regenerative anem ia is fundamental. W ith identical low Hb. concentration a patient is able to live norm ally (simple aplastic disease) or his activities are limited by easy fatigability (hyperactive erythropoiesis) (fig. 1). In adults, bone marrow volume is about 2.600 cc. (2), equal to 5/9 of blood volume. Normally, h alf of it is in activity with 6% of nucleated cells (3). Fat marrow can be replaced in a few days (4) due to the regenerative potential of hemopoiesis. C r o s b y a n d A k e r o y d (5) calculated that as

Journal

Acta HaematologicaKarger

Published: Jan 1, 2009

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