This book is a wide-ranging guide to the diagnosis and management of the numerous medical and surgical complications that may arise in patients with sickle cell anemia. After introductory chapters on the genetics, pathophysiology, clinical features, and variants of sickle cell anemia, the complications observed in different parts of the body are addressed in a series of well-illustrated chapters. The coverage includes splenic, hepatobiliary, musculoskeletal, gastrointestinal, ophthalmological, cardio- and cerebrovascular, and renal complications, as well as acute chest syndrome, leg ulcers, hand and foot syndrome, acute appendicitis, and priapism. Treatment-oriented chapters consider perioperative management, blood transfusion therapy, hydroxyurea treatment, hematopoietic stem cell transplantation, and emerging strategies. The book is clearly written in a distinctive bullet point format for ease of reference and emphasizes especially aspects of practical significance. It will be of value for hematologists, general surgeons, internists, pediatricians, pediatric surgeons, fellows, residents, medical students, and nurses. Abstract source

Sickle Cell Disease is the most common genetic disease world wide and in the UK. It has marked geographical variation in its distribution in the UK, with a concentration in London and other major conurbations (Birmingham and Manchester). In these areas, specialist centres have become established offering expert, up to date care for both inpatients and out patients with Sickle Cell Disease. Although patient numbers are increasing outside these areas, the expertise of health professionals can be patchy. This book aims to provide a user friendly, accessible resource for areas with smaller numbers of patients, to allow them to provide equitable care with the larger well established centres. Sickle Cell Disease can be associated with acute life threatening complications, when clear, easily available advice is needed, and with chronic long term complications which may need liaison with other health professionals. Clear treatment protocols for all the common complications of sickle cell disease, are outlined here, with summaries of key evidence and references. Abstract source

Sickle Cell Pain is a panoramic, in-depth exploration of every scientific, human, and social dimension of this cruel disease. This comprehensive, definitive work is unique in that it is the only book devoted to sickle cell pain, as opposed to general aspects of the disease. The 752-page book links sickle cell pain to basic, clinical, and translational research, addressing various aspects of sickle pain from molecular biology to the psychosocial aspects of the disease. Supplemented with patient narratives, case studies, and visual art, Sickle Cell Pain’s scientific rigor extends through its discussion of analgesic pharmacology, including abuse-deterrent formulations. The book also addresses in great detail inequities in access to care, stereotyping and stigmatization of patients, the implications of rapidly evolving models of care, and recent legislation and litigation and their consequences. Abstract source

Sickle Cell Anemia with Splenic Sequestration: II. Epidemiology Most common in ages 1 to 4 years old III. Pathophysiology A. Splenic infarction typically occurs by Puberty in patients with Hemoglobin SS B. However, less severe forms of Sickle Cell Disease can occur in adulthood IV. Precautions Rapidly progressive course with significant risk of decompensation secondary to acute Anemia V. Signs A. Anemia B. Abdominal Pain C. Splenomegaly 1. Acute splenic enlargement (especially in under age 5 years, before auto-infarction) 2. Contrast with Sickle Cell Hemolytic Crisis in which the Spleen will be small or non-palpable Abstract source

Sickle Cell Anemia: II. Epidemiology A. Sickle Cell Disease (Sickle Cell Anemia) Prevalence (U.S.): 100,000 (1 per 365 black or african american descent) B. Sickle Cell Trait (A/S) Incidence 1. Americans of African Descent: 1 in 12 2. Also Seen in Greeks, Italians, Turks, Saudi Arabians and hispanic patients III. Pathophysiology A. Normal Hemoglobin A replaced by Hemoglobin S (Hb S) Deoxygenated Hemoglobin-S assumes a sickle shape B. Substitution of Valine for glutamic acid Occurs at the 6th position of the beta-chain C. Mechanisms of dysfunction 1. Hypercoagulable a. Round blood cells b. Clotting Cascade activation c. Inflammatory cascade activation d. Platelet activation Abstract source

Cerebrovascular Accident in Sickle Cell Anemia: II. Epidemiology: Prevalence Children: 5% Highest risk at ages 2 to 5 years old with Sickle Cell Anemia A. Silent cerebral infarct occurs in up to 25% of children by age 6 years old B. Young adult (age <20): 11% C. Age 45 years old: 24% D. Lifetime risk: 25% III. Pathophysiology A. Silent Cerebral Infarction 1. Silent CVA may occur in up to 25% of children with Sickle Cell Anemia by age 6 years old 2. May present as cognitive deficits or learning difficulties 3. May be prevented with regular Blood Transfusions (see below) B. Ischemic Stroke 1. Most common in children and teens 2. Internal Carotid Artery thrombosis C. Hemorrhagic Stroke 1. Most common in ages 20 to 30 years old Abstract source

Sickle cell disease (SCD), an inherited hemolytic anemia, is associated with multiple acute and chronic complications such as painful vasoocclusive events, cerebral vasculopathy, priapism, and renal or lung disease. These complications are variable and unpredictable, and can be associated with significant morbidity and poor quality of life. This book covers several areas regarding pathology, diagnosis, complications, signs, symptoms and medical treatments. There are few studies in literature on the role of physiotherapy as a resource to prevent and treat locomotor system disorders, respiratory problems and painful crises in SCD individuals. This book highlights the role of physiotherapy in sickle cell anemia. A comprehensive and authoritative monograph, this book will be equally interesting to both established researchers and to graduate students interested in both genetics and the physical therapy field. Abstract source

Blood transfusions are an important part of hematologic care. This issue of Hematology/Oncology Clinics will focus on transfusion medicine and will include articles on: RBC Transfusions: Conclusions from Clinical Trials and the Establishment of Evidence-based Guidelines for Adults, Platelet Transfusions: Conclusions from Clinical Trials and the Establishment of Evidence- and/or Experience-based Guidelines for Adults, Use and Overuse of Plasma Products: Establishment of Evidence- and/or Experience-based Guidelines for Plasma Transfusion in Adults, Stem Cell Mobilization/Collection: Coordination Between Hem/Onc, Transplant, and Transfusion Services, Management of Patients with Sickle Cell Disease Using Transfusion Therapy: Guidelines and Complications, and many more exciting articles. Abstract source

Sickle cell anemia is a multisystem disease associated with episodes of acute illness and progressive organ damage. Hemoglobin polymerization, leading to erythrocyte rigidity and vasoocclusion, is central to the pathophysiology of the disease, but the importance of chronic anemia, hemolysis, and vasculopathy has been established. The most common cause of sickle cell anemia is the HbS variant (141900.0243), with hemoglobin SS disease being most prevalent in Africans(review by Rees et al., 2010). Piel et al. (2017) reviewed the genetic and nongenetic modifiers of the severity of sickle cell disease. Scriver and Waugh (1930) reported detailed studies of a 7-year old child with sickle cell anemia. Her main complaints were cough, night sweats, vague pains in the legs and joints, occasional abdominal pain, poor appetite, and increasing fatigue. In a series of clever experiments that involved taking venous blood from the arm under different circumstances, the authors showed a correlation between oxygen tension and sickling of the red blood cells in vivo. Increased sickling was observed when oxygen pressure fell below 40 to 45 mm Hg. Scriver and Waugh (1930) concluded that large aggregations of sickle cells seen in sinuses, vessels, and organs of sickle cell patients at autopsy reflected lowered oxygen tension resulting from death. Abstract source

Although sickle cell anemia was the first molecular disease to be identified, its complex and fascinating pathophysiology is still not fully understood. A single mutation in the beta-globin gene incurs numerous molecular and cellular mechanisms that contribute to the plethora of symptoms associated with the disease. Our knowledge regarding sickle cell disease mechanisms, while still not complete, has broadened considerably over the last decades. Sickle Cell Anemia: From Basic Science to Clinical Practice aims to provide an update on our current understanding of the disease’s pathophysiology and use this information as a basis to discuss its manifestations in childhood and adulthood. Current therapies and prospects for the development of new approaches for the management of the disease are also covered. Abstract source