By Hubert Schrezenmeier, Andrea Bacigalupo
This booklet takes account of the latest findings in laboratory learn and scientific trials to supply a entire and up to date reference at the pathophysiology, epidemiology, analysis and remedy of received and inherited aplastic anemia. in addition to delivering a close evaluation of the pathophysiology of the illness, the foreign workforce of authors covers all features of administration, together with the proven methods of bone marrow transplantation and immunosuppressive remedy, new techniques corresponding to using hematopoietic progress elements and escalated immunosuppression, and arguable matters comparable to stem cellphone transplantation. additionally integrated is a crucial foreign consensus rfile on remedy, and a last part concentrates at the inherited syndrome Fanconi's anemia. special remedy guidance are given, making this the definitive source for hematologists and different clinicians occupied with the administration and supportive care of sufferers with aplastic anemia. Scientists attracted to bone marrow failure also will locate this a useful reference.
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Extra resources for Aplastic Anemia: Pathophysiology and Treatment
Harada, M. and Niho, Y. (1992) Levels of human serum granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor under pathological conditions. Biotherapy, 4, 147–53. Rusten, L. , Lyman, S. , Veiby, O. P. and Jacobsen, S. E. W. (1996) The ﬂt-3 ligand is a direct and potent stimulator of the growth of primitive and committed human CD34ϩ bone marrow progenitor cell in vitro. Blood, 87, 1317–25. de Sauvage, F. , Hass, P. , Spencer, S. , Malloy, B. , Gurney, A. , Spencer, S.
Longmore, G. , Klingler, K. and Johnson, G. R. (1995) Lineage-restricted recruitment of immature haematopoietic cells in response to erythropoietin after normal haematopoietic cell transfection with erythropoietin receptor. Experimental Haematology, 23, 645. Metcalf, D. (1988) In The molecular control of blood cells. London: Harvard University Press. , Slanicka-Krieger, M. et al. (1995) Persistent growth impairment of bone marrow stroma after antilymphocyte globulin treatment for severe aplastic anaemia and its association with relapse.
Erythropoietin (EPO) EPO is a glycoprotein produced by cells adjacent to the proximal renal tubules (Erslev, 1991; Kranz, 1991). In the bone marrow, EPO binds to and activates speciﬁc receptors on erythroid progenitor cells to regulate the production of red blood cells (RBCs). Under normal steady-state conditions, 10 to 20 mIU/ml of plasma EPO induces the production of enough RBCs to replace senescent cells in the RBC mass, and to maintain a ﬂow of oxygen to the renal oxygen sensor which is suﬃcient to ensure baseline levels of EPO synthesis.