Disease Knowledge Centres

  • Haematology - Disease Topic Overview

    Haematology encompasses the study of blood formation (haemopoiesis) and function, as well as the diagnosis and treatment of diseases of the blood.1

    Anaemia is a disease that causes an abnormally low erythrocyte cell mass, either by reduced production or increased break down of these cells.2 The most common cause of anaemia is a lack of iron, causing reduced production of haemoglobin and reduced oxygen distribution. This is easily treated with iron supplements and a healthy balanced diet.3 It is a disease in its own right, but it is also associated with many others (heart disease,4,5 cancer6 and diabetes7).

    Sickle cell anaemia is an autosomal recessive disease which causes structural mutations in haemoglobin molecules and sickle shaped erythrocytes.8 There is a high prevalence of sickle cell anaemia among the Afro Caribbean population. It can cause pulmonary complications such as acute chest syndrome and sickle chronic lung disease.9 The high morbidity and mortality associated with this disease make it public health issue.

    Genetic mutations in coagulation factors can lead to coagulation disorders which cause excessive bleeding. Haemophilia, caused by a mutation in the factor VIII gene, is the most common of these disorders; however deficiencies of other coagulation factors can also occur.10

    Leukemias and lymphomas are both classified as cancers of the blood; they affect white blood cells and the lymphatic system respectively. There are four main types of leukaemia; acute lymphoblastic leukaemia, acute myeloid leukaemia, chronic myeloid leukaemia and chronic lymphocytic leukaemia.11 Lymphomas can be divided into Hodgkin's lymphomas (HL), and non-Hodgkin's lymphomas (NHL). Both form neoplasms in the lymphatic system, NHL are more prevalent and can be further separated into B-cell or T/NK-cell neoplasms.12

    1. Hoffbrand A.V. et al. Essential Haematology. Wiley-Blackwell. 2006 ; 5 (698) : viii.
    2. Uthman E. Understanding Anemia. Understanding Health and Sickness Series. Univ. Press of Mississippi. 1998 : 3-10.
    3. Jacoby D.B. et al. Encyclopedia of Family Health. Marshall Cavendish. 2004 ; 5 : 88-90.
    4. Dimopoulos K. et al. Anemia in Adults With Congenital Heart Disease Relates to Adverse Outcome. Journal of the American College of Cardiology. November 2009 ; 54 (22) : 2093-2100.
    5. Teng K.T.-H. et al. Mild Anaemia is Associated with Increased All-cause Mortality in Heart Failure. Heart, Lung and Circulation. January 2010 ; 19 (1) : 31-37.
    6. Ludwig H. et al. The European Cancer Anaemia Survey (ECAS): A large, Multinational, Prospective Survey Defining the Prevalence, Incidence, and Treatment of Anaemia in Cancer Patients. European Journal of Cancer. October 2004 ; 40 (15) : 2293-2306.
    7. McGill H.B. et al. Anemia and the Role of Erythropoietin in Diabetes. Journal of Diabetes and its Complications. July-August 2006 ; 20 (4) : 262-272.
    8. Peterson J.M. Sickle Cell Anaemia. The Rosen Publishing Group. 2008 : 15-25.
    9. Greenough A. et al. Systemic Disease Sickle Cell Disease. Encyclopedia of Respiratory Medicine. May 2006 : 212-218.
    10. Hoffbrand A.V. et al. Essential Haematology. Wiley-Blackwell. 2006 ; 5 (698) : 290-296.
    11. Basso G. et al. Diagnosis and Genetic Subtypes of Leukemia Combining Gene Expression and Flow Cytometry. Blood Cells, Molecules and Diseases. September-October 2008 ; 39 (2) : 164-168.
    12. Ottensmeir C. The Classification of Lymphocytes and Leukaemias. Chemico-Biological Interactions. June 2001 ; 135-136 : 653-664.

Latest Multi Media

An Animation Explaining the Mechanisms Involved in Hereditary Spherocytosis

Haematology Drug Data - A-Z English

Drug Updates

Fenofibrate 267mg Capsules
Fenofibrate is indicated as an adjunct to diet and other non-pharmacological treatment (e.
Ferriprox 100 mg/ml oral solution
Ferriprox is indicated for the treatment of iron overload in patients with thalassaemia major when deferoxamine therapy is contraindicated or inadequate.
Ferriprox 500 mg film-coated tablets
Ferriprox is indicated for the treatment of iron overload in patients with thalassaemia major when deferoxamine therapy is contraindicated or inadequate.

Latest Drug News

Nordic rights to ReFacto and BeneFIX are returned to Pfizer - 17-02-2012
Pfizer has taken back the Nordic territory rights to reclaim to the blood clotting factors ReFacto and BeneFIX from Swedish Orphan Biovitrum which held co-promotion rights there.The drugs will continue to be manufactured in Sweden.
Early results for SM101 (Suppremol) are encouraging for ITP - 16-02-2012
Interim results from a Phase 1b/IIa study of SM 101 from Munich-based SuppreMol GmbH for the treatment of primary Immune Thrombocytopenia were encouraging. SM 101 is a recombinant, soluble, non glycosylated version of the Fc receptor IIb. The protein binds to autoantibody/autoantigen complexes and blocks the activation of Fc receptors on the surface of immune cells. As a result, the immune response is down regulated and triggering of the inflammation cascade is prevented. The drug is also in an early trial for SLE.

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Latest Clinical Trials

Effects of Mixed Exercise Regime and L-Carnitine Supplementation in HIV Patients on HAART
HIV patients treated with Highly Active AntiRetroviral Therapy (HAART) show significant metabolic symptoms, such as lipodystrophy, dyslipidemia, and insulin resistance. A possible contribution to these symptoms in HIV/HAART is a decrease in mitochondrial function, resulting in a decreased fatty acid oxidation. A combined regime of aerobic and resistance training has been demonstrated to increase lean body mass and reduce overall fat and truncal fat and the levels of triglyceride and LDL cholesterol.
HIV Vaccine Study in HIV Positive Patients
The purpose of the study is to see whether a single vaccination (injection) with the investigational HIV vaccine is safe and effective in patients who are HIV positive but have not yet begun anti-retroviral therapy. As this is an exploratory study, four different dose formulations of HIV vaccine will be investigated. This study will evaluate whether or not the HIV vaccine is able to reduce the HIV viral load (number of HIV virus particles in the blood) and increase or slow the decline in CD4 T cell count.

Latest Journal Publications

Palliative Medicine
Haematological malignancies are complex diseases, affecting the entire age spectrum, and having marked differences in presentation, treatment, progression and outcome. Patients have a significant symptom burden and despite treatment improvements for some sub-types, many patients die from their disease. We carried out a systematic review and meta-analysis to examine the proportion of patients with haematological malignancies that received any form of specialist palliative or hospice care. Twenty-four studies were identified, nine of which were suitable for inclusion in the meta-analysis. Our review showed that patients with haematological malignancies were far less likely to receive care from specialist palliative or hospice services compared to other cancers (Risk Ratio 0.46, [95% confidence intervals 0.42–0.50]). There are several possible explanations for this finding, including: ongoing management by the haematology team and consequent strong bonds between staff and patients; uncertain transitions to a palliative approach to care; and sudden transitions, leaving little time for palliative input. Further research is needed to explore: transitions to palliative care; potential unmet patient needs; where patients want to be cared for and die; existing practices in the delivery of palliative and end-of-life care; and barriers to specialist palliative care and hospice referral and how these might be overcome.
British Journal of Haematology
The goal of platelet transfusions is to prevent severe and life-threatening bleeding in patients with thrombocytopenia. This aim needs to be balanced against the risks associated with platelet transfusions as well as the challenge of maintaining an adequate supply. This review summarizes the recent evidence regarding the clinical use of platelet transfusions in haematology patients, concentrating on the topics that still continue to provoke debate. These include the optimal dose for platelet transfusions and the relative safety of a ‘therapeutic only’ platelet transfusion strategy compared to the use of prophylactic platelet transfusions. The type of platelet product has been the subject of two recent systematic reviews. The results of these reviews will be discussed as well as their implications for current practice.

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Haematology