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CDHB

Haemorrhagic Disorders

Platelet disorders usually result in surface bleeding such as epistaxis and petechiae. Coagulation disorders produce deep bleeding such as haemarthroses or muscle haematomas. There may be a mixed pattern of bleeding in DIC. Fatal intracranial haemorrhage may occur in either severe thrombocytopaenia, platelet dysfunction or a severe coagulation deficiency.

Investigation of a patient presenting with a possible haemorrhagic disorder

• Family history, history of pattern of bleeding, recent drugs, dietary history, possibility of HIV.
• CBC + diff, ESR or CRP, blood film examination.
• Prothrombin time, partial thromboplastin time, thrombin time and fibrinogen level. Use citrate tubes. Take care to add the correct amount of blood to these tubes and avoid heparin contamination from heparin containing IV lines, blood tubes, etc. Take blood samples before any transfusions are given.

  Note: These are only screening tests and do not necessarily exclude defects which may result in abnormal bleeding. Consultation with the Coagulation Laboratory is strongly recommended ( 80374).

Treatment

• This is entirely dependent on the results of the initial tests obtained. If a severe thrombocytopaenia (platelets <10 x 10⁹/L) is present then this constitutes a medical emergency. An accurate diagnosis is necessary and this will often require bone marrow examination. These patients may need platelet transfusions.
• Patients with known coagulation defects (Haemophilia A, Haemophilia B, Von Willebrand’s disease, etc.) present special problems and consultation (day or night) is essential when these patients are admitted outside the Haematology Service. Patients with an established coagulation defect may carry a card giving essential details of their condition. Those living around Christchurch will have records available in the Haematology Department, Haemostasis office and Haematology Ward, giving the relevant Factor levels and some clinical details. Always take a suspected bleed seriously; always take careful note of any advice the patient gives you. Always contact a Haematologist or the Haemostasis Nurse.
• Refer to the Haematology Department Protocols and Guidelines (online at http://redbook.streamliners.co.nz) for management of haemophilia, including local practice (these guidelines are based on the New Zealand National Guidelines for the Management of Haemophilia).
• In haemophilia A life-threatening bleeding requires immediate Factor VIII infusion, with concentrated freeze-dried preparations, e.g., CSL Factor VIII Biostate or recombinant factor VIII. A rough guide is given by the following formula.

• You will need to know what level of Factor VIII it is desirable to achieve in any particular clinical situation (see above formula). Round to the nearest vial. Do not throw any product away. Every effort should be taken to ensure each patient receives the same specific concentrate that the patient has recently been using.
• In Von Willebrand's disease and mild haemophilia A, desmopressin or CSL Factor VIII concentrate (Biostate) is used. Desmopressin may be given in a dose of 0.3 microgram/kg in 50 mL sodium chloride 0.9% IV over 30 minutes (starting 60 minutes pre-op, if requiring surgery). Desmopressin can be given undiluted subcut. Mild haemophilia A patients who infrequently use coagulation factor concentrates, and for whom DDAVP is contraindicated, should receive recombinant products.
• In Haemophilia B (Christmas disease, factor IX deficiency) Factor IX concentrate (Monofix) or the recombinant product (Benefix) is the treatment of choice. Consult Haematologist for this and less common coagulation disorders.

Topic Code: 1444