By Drew Provan
Masking hematological facets of parts resembling pathology, molecular technology, melanoma, and common medication, this consultant comes in handy as a reference for normal perform and medical institution employees, hematologists and trainees in hematology. This moment variation displays advances within the knowing of the molecular biology of affliction because the first version used to be released in 1998. Provan teaches within the division of Hematology at Queen Mary's tuition of drugs and Dentistry.
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Extra info for ABC of Clinical Haematology
On admission to a specialist unit the patient will need chemotherapy to treat the leukaemia and supportive care to ameliorate or correct the effects of the leukaemia and to facilitate treatment. 5 Interphase fluorescent in situ hybridisation using probes for BCR and ABL genes. Left: Normal cell showing two red dots (two normal copies of BCR) and two yellow dots (two normal copies of ABL). 4 Management of acute leukaemia • • • • • • Immediate (same day) referral to specialist Prompt diagnosis Early treatment Intensive supportive care Systemic chemotherapy Treatment directed at central nervous system (in children and in adult acute lymphoblastic leukaemia) • Minimising early and late toxicity of treatment Adequate hydration and allopurinol are essential at the start of treatment to reduce the risk of hyperkalaemia, hyperuricaemia, and renal damage Supportive care The numerical threshold for blood product transfusion has progressively lowered.
In platelet storage pool diseases deficiencies in either the ␣ or dense granules cause poor secondary platelet aggregation. There are also a variety of further specific surface membrane defects and internal enzyme abnormalities, which although difficult to define, can cause troublesome chronic bleeding problems. Acquired abnormalities Decreased production of platelets due to suppression or failure of the bone marrow is the commonest cause of thrombocytopenia. In aplastic anaemia, leukaemia and marrow infiltration, and after chemotherapy, thrombocytopenia is usually associated with a failure of red and white cell production but may be an isolated finding secondary to drug toxicity (penicillamine, cotrimoxazole), alcohol, or viral infection (HIV, infectious mononucleosis).
Acquired disorders In thrombocytopenia due to bone marrow failure or marrow infiltration—for example leukaemia or cancer—prophylactic platelet transfusions are given to keep the platelet count above 10 ϫ 109/l though the threshold is higher in infected or bleeding patients or to cover invasive procedures. 6 Treatment of platelet disorders Congenital disorders • Platelet transfusions (leucodepleted, HLA compatible and irradiated) • DDAVP • Tranexamic acid • Recombinant factor VIIa • Bone marrow transplantation Acquired disorders • Bone marrow failure Platelet transfusions if platelet count Ͻ10 ϫ 109/l • Idiopathic thrombocytopenic purpura (adults) Prednisolone Intravenous immunoglobulin Splenectomy • Post-transfusion purpura Intravenous immunoglobulin Plasma exchange • Heparin-induced thrombocytopenia Anticoagulation but without heparin • Thrombotic thrombocytopenic purpura Large volume plasma exchange Aspirin when platelets Ͼ50 ϫ 109/l • Disseminated intravascular coagulation Treat underlying cause Fresh frozen plasma Platelet transfusion • Hypersplenism Splenectomy if severe • Platelet function disorders Platelet transfusion DDAVP (occasionally of use; for example in uraemia) 31 ABC of Clinical Haematology in life-threatening bleeding.