FVIII/FIX Prophylaxis for Severe Hemophilia
Experience with clotting factor concentrate (CFC) replacement products over several decades has shown that regular replacement (prophylaxis) is the only way to prevent musculoskeletal damage in hemophilia and impact the natural history of hemophilia. Yet there is lack of data on the optimal age to start such replacement therapy and the regimens to be used. While very early administration of high doses are certainly more effective in preventing bleeding, cost and compliance is a major constraint all over the world. (Source: Seminars in Hematology)
Source: Seminars in Hematology - October 26, 2015 Category: Hematology Authors: Manuel Carcao, Alok Srivastava Source Type: research
Hemophilia in the 21 Century: Tremendous Progress, Tremendous Opportunity
Since antiquity, when a recognition of a familial, fatal bleeding disorder in males was first recorded,1 the world has been inhabited by individuals who lack adequate activity of coagulation factor VIII (FVIII) or IX (FIX). It was not until 1803, however, that Dr. Joseph Conrad Otto, a physician in the United States, published the first description of a case of a heritable bleeding condition transmitted through mothers to some of their sons.2 Eventually, in additional to spontaneous and traumatic bleeding, the disorder was understood to be distinguished phenotypically by a singular and distinctive tendency for bleeding int...
Source: Seminars in Hematology - October 26, 2015 Category: Hematology Authors: Patrick F. Fogarty Source Type: research
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Source: Seminars in Hematology - September 22, 2015 Category: Hematology Source Type: research
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Source: Seminars in Hematology - September 22, 2015 Category: Hematology Source Type: research
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Source: Seminars in Hematology - September 22, 2015 Category: Hematology Source Type: research
Non-Immune Hemolysis: Diagnostic Considerations
Non immune hemolytic anemia (NIHA) is characterised by positive routine hemolytic tests but negative antihuman immunoglobulin (Coombs) test. Hereditary non immune hemolysis includes disorders of erythrocytic enzymes, membrane, hemoglobin (qualitative and quantitative disorders) as well as the rare hereditary forms of thrombotic microangiopathies. Acquired NIHA includes PNH, infections, drug and metal intoxications with as a target red blood cells or endothelium of capillaries, the rare acquired forms of thalassemia or erythrocytic membrane disorders, hemolysis secondary to a dysfunctioning artificial (prosthetic) cardiac v...
Source: Seminars in Hematology - July 20, 2015 Category: Hematology Authors: Photis Beris, Véronique Picard Source Type: research
Anemia in Clinical Practice—Definition and Classification: Does Hemoglobin Change With Aging?
Anemia is a global public health problem affecting both developing and developed countries at all ages. According to the World Health Organization (WHO), anemia is defined as hemoglobin (Hb) levels (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 17, 2015 Category: Hematology Authors: M. Domenica Cappellini, Irene Motta Source Type: research
Anemia in Clinical Practice. Definition and classification. Does Hb change with aging?
Anemia is a global public health problem affecting both developing and developed countries at all ages. According to WHO, anemia is defined as hemoglobin (Hb) levels (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 17, 2015 Category: Hematology Authors: M. Domenica Cappellini, Irene Motta Source Type: research
Seminars Bids Adieu and αντίο to Photis Beris
Professor Photis Beris will step down from his position as Co-Editor of Seminars in Hematology at the end of 2015. Professor Beris has served in this leadership role since 2000, but his relationship with the journal is even older, as a young assistant to founding Editor Peter Miescher. (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 17, 2015 Category: Hematology Authors: Neal Young Source Type: research
Iron Deficiency Anemia in Pregnancy
Anemia is a common problem in obstetrics and perinatal care. Any hemoglobin below 10.5 g/ dL can be regarded as true anemia regardless of gestational age. Reasons for anemia in pregnancy are mainly nutritional deficiencies, parasitic and bacterial diseases and inborn red cell disorders such as thalassemias. Main cause of anemia in obstetrics is iron deficiency, which has a worldwide prevalence between estimated 20-80 % of especially female population. Stages of iron deficiency are depletion of iron stores, iron deficient erythropoiesis without anemia and iron deficiency anemia, the most pronounced form of iron deficiency. ...
Source: Seminars in Hematology - July 10, 2015 Category: Hematology Authors: Christian Breymann Source Type: research
ANEMIA OF CHRONIC DISORDERS: New Diagnostic Tools and New Treatment Strategies
Anemia in the setting of chronic inflammatory disorders is a very frequent clinical condition which is, however, often neglected or not properly treated given the problems often caused by the diseases underlying the development of anemia. Mechanistically, anemia is mainly caused by inflammation driven retention of iron in macrophages making the metal unavailable for heme synthesis in the course of erythropoiesis, further by an impaired biological activity of the red blood cell hormone erythropoietin and a reduced proliferative capacity of erythroid progenitor cells. (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 10, 2015 Category: Hematology Authors: Guenter Weiss Source Type: research
Anemia in Clinical Practice: Introduction
Anemia is the most frequent pathology in humans. It is seen in all ages with a lower incidence in men between the ages of 10 and 50 years (Figure 1).1 Anemia may be a unique disease; however, it is frequently associated with other pathologic conditions, so that almost all medical specialists have to deal with anemia of different degrees and etiologies. In fact, there is no physician that has never been confronted with an anemic patient. In certain conditions, the presence or absence of anemia modifies the prognosis (see reviews on anemia in myelodyplastic syndromes [MDS] and anemia in the elderly in this issue). (Source: S...
Source: Seminars in Hematology - July 9, 2015 Category: Hematology Authors: Maria-Domenica Cappellini, Photis Beris Source Type: research
Anemia in clinical practice
Anemia is the most frequent pathology in humans. It is seen in all ages with a lower incidence in men between 10 and 50 years (Fig 1, Ref 1). Anemia may be the unique disease; however frequently is associated with other pathologic conditions, so that almost all medical specialists have to deal with anemia of different degree and etiology. In fact there is no physician that has never been confronted with an anemic patient. In certain conditions the presence or absence of anemia modifies prognosis (see anemia in MDS in the present issue and anemia in the elderly). (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 9, 2015 Category: Hematology Authors: Maria-Domenica Cappellini, Photis Beris, Photis Beris Source Type: research
Anemia of Central Origin
Hypoproliferative anemia results from the inability of bone marrow to produce adequate numbers of red blood cells. The list of conditions that cause hypoproliferative anemia is long, starting from common etiologies as iron deficiency to rarer diagnoses of constitutional bone marrow failure syndromes. There is no perfect diagnostic algorithm, and clinical data may not always clearly distinguish “normal” from “abnormal”, yet it is important for practicing clinicians to recognize each condition so that treatment can be initiated promptly. (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 8, 2015 Category: Hematology Authors: Kazusa Ishii, Neal S. Young Source Type: research
Anemia as the Main Manifestation of Myelodysplastic Syndromes
Myelodysplastic syndromes (MDS) are a constellation of different diseases sharing anemia in the great majority of cases, and this cytopenia defines these pathologies and their most dramatic clinical manifestations. Anemia in MDS is due to ineffective erythropoiesis, with a high degree of apoptosis of marrow erythroid progenitors. These progenitors show distinctive dysplastic features that consent diagnosis, and are recognizable and differentiated, although not easily, from other morphologic alterations present in other types of anemia. (Source: Seminars in Hematology)
Source: Seminars in Hematology - June 30, 2015 Category: Hematology Authors: Valeria Santini Source Type: research
