Titlebook: Hereditary Diseases and Blood Transfusion; Proceedings of the N C. Th. Smit Sibinga,P. C. Das,E. Briët Conference proceedings 1995 Springer

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The Current Status of Bone Marrow Transplantation and Gene Therapy in the Management of the Haemogloaemoglobinopathies. To date the procedural risks of BMT have precluded its widespread use for thalassaemiaand sickle cell disease. However as the risks of BMT have decreased due to a combination of increasing experience and improved techniques, it has become a viable alternative to medical treatment

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The Molecular Biology of Blood Groups: Relevance to the Study of Erythroid Maturation and the Clinicrent blood group systems found on human red cells. Two of these systems (CH/RG, LE) are not intrinsic to the red cell but acquired from plasma. Four systems (ABO, H, P, LE) are defined by carbohydrate structures and cDNAs corresponding to the glycosyl transferase responsible for ABO, H and LE antige

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Genetic Abnormalities in Blood Group Serologydentification of blood groups whose expression is genetically determined. Blood group antigens are polymorphic, inherited, structural characteristics that are located on proteins, glycoproteins or glycolipids on the exofacial surface of the RBC membrane.

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Translocation in Cancer: Mechanism of Oncogenic Conversion and Implications for Therapy the molecular characterization of the translocation involving the c-MYC gene in Burkitts lymphoma, our understanding of the pathways by which genetic translocations in haematologic malignancy lead to oncogenesis has increased dramatically. Molecular studies of the genes involved in these somatic re

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Role of Topoisomerase II , and , Isozymes in Determining Drug Resistance , and ,s. Treatments commonly include an anthracycline(e.g. doxorubicin) or anthracenedione (e.g. mitoxantrone), and, more recently, regimens have also included amsacrine (m-AMSA) [1] or etoposide [2]. Complete responses occur in 50–57% of patients. However, the underlying biochemical factors which disting

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Impact of Cell Culture Technology on Transfusion Medicinem normal mice could re-instate haematopoiesis in lethally irradiated mice [1]. The concepts of a multipotent haematopoietic cell emerged from these early experiments, and led to the development of . investigative tools to help identify the cell(s) responsible for initiating and maintaining haematopo

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Gene Therapy for Primary Immune Deficiencies first successful histocompatible bone marrow transplants (BMT), haploidentical T-cell depleted BMT, or matched unrelated donor BMT [1]. Because patients with SCID have defective immunity, their ability to reject transplanted marrow is limited. This characteristic has made it possible to achieve eng

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Molecular Basis of Thrombophiliain-, protein C-, and protein S genes were the best characterized hereditary risk factors for venous thrombosis. This chapter will focus on recent developments in understanding abnormalities in the protein C anticoagulant pathway [for reviews see refs 1–3].