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Postgraduate Haematology provides up-to-date knowledge of the pathogenesis, clinical and laboratory features, management and treatment of a wide range of blood and bone marrow disorders in a concise and user friendly style.
- Up-to-date knowledge of the pathogenesis, clinical and laboratory features and management of all blood disorders
- New sections reflect advances in the specialty, including knowledge gained from new generation sequencing, latest anticoagulant drugs, diagnostic laboratory tools, and treatment strategies
- Superb four-color illustrations and photomicrographs of blood cells and tissues throughout
- Includes algorithms to aid with decision-making for treatment
- Companion website includes figures and tables for download
long-term risks after HSCT are infertility and second malignancy. Selection of candidates for HSCT is complex owing to the uncertain long-term course of the disease. HSCT should be considered in children (age <16 years) with SCD (any genotype) who have a human leucocyte antigen (HLA)-identical related donor and evidence of target organ damage involving the brain, lungs, kidneys or eyes. Children who are placed on longterm blood transfusions for any indication should also be evaluated for HSCT.
erythroid cells. Its binding sites are found in the regulatory elements of many erythroid-specific genes, including the β-globin gene. All of these transcription factors play key roles in coordinating erythroid maturation and globin gene regulation. Mutations of GATA1 and KLF1 are rare, but have been described in families with abnormalities of haemoglobin synthesis (Chapter 6), disorders of the red cell membrane (Chapter 8), abnormal haem synthesis (Chapter 3) and other abnormalities of
through the ileum in humans and is efficient for small (a few micrograms) oral doses of cobalamin. This is the normal mechanism by which the body acquires cobalamin and is mediated by gastric intrinsic factor (IF). Dietary cobalamin is released from protein complexes by enzymes in the stomach, duodenum and jejunum; it combines 59 Postgraduate Haematology Mol wt = 56,000–58,000 Source: granulocytes ? other tissues TCl + III (HCs) 800 ng/L 450 ng/L 30–40 ng/L Cobalamin then enters the ileal
has also been described in association with H. pylori infection, long-term use of histamine H2 -receptor antagonists and protonpump inhibitors, chronic alcoholism, pancreatic exocrine failure, Sj¨ogren syndrome and systemic sclerosis. The syndrome is associated with low serum cobalamin levels, with or without evidence of cobalamin deficiency, such as raised serum levels of methylmalonic acid and homocysteine. A minority of patients with food cobalamin malabsorption may go on to develop clinically
in rare instances. Pigmented gallstones are seen in two-thirds of patients, particularly those with HbSS, and can occur in young children. Patients with abdominal symptoms attributable to gallstones should undergo cholecystectomy, although the management of asymptomatic gallstones is less clear. Laparoscopic cholecystectomy can be safely performed, but associated common duct bile stones first require endoscopic retrograde cholangiopancreatography. As patients with sickle cell disease are living