Australian Red Cross Blood Service

Absence/low levels of coagulation factor VIII.

Clinical severity is determined by the factor VIII level:

• Severe (<1% factor VIII)
  • spontaneous and delayed deep soft tissue bleeding, particularly into joints and muscles
• Moderate (1%–5% factor VIII)
  • severe bleeding after mild trauma
• Mild (6%–25% factor VIII)
  • severe bleeding when tissues are damaged by surgery or trauma

The minimal effective level for haemostasis is generally about 25%–30% factor VIII level.

Desmopressin (DDAVP) for mild Haemophilia A (baseline factor VIII above 15%).

Replacement therapy with recombinant factor VIII (Kogenate and Xyntha) or plasma-derived concentrates (Biostate).

Consultation with a haematologist is recommended.

Patients are usually registered with and cared for by a Specialist Haemophilia Treatment Centre, who should be contacted when a patient presents to another clinical unit or for immediate specialist assistance (eg, when head injury is suspected).

Related topics

Evidence-based clinical practice guidelines for the use of recombinant and plasma-derived FVIII and FIX products [on www.nba.gov.au]

Australian Bleeding Disorders Registry and Haemophilia Treatment Centre Contact list [on www.ahcdo.org.au]

Back to top

Absence/low levels of coagulation factor IX.

Replacement therapy with recombinant factor IX (Benefix) or plasma-derived concentrates (MonoFIX-VF).

Prothrombinex-VF is no longer the preferred plasma-derived product of choice for haemophilia B; however a small number of patients remain on this product.

Consultation with a haematologist is recommended.

Patients are usually registered with and cared for by a Specialist Haemophilia Treatment Centre, who should be contacted when a patient presents to another clinical unit or for immediate specialist assistance (eg, when head injury is suspected).

Related topics

Evidence-based clinical practice guidelines for the use of recombinant and plasma-derived FVIII and FIX products [on www.nba.gov.au]

Australian Bleeding Disorders Registry and Haemophilia Treatment Centre Contact list [on www.ahcdo.org.au]

Back to top

Development of autoantibodies directed against von Willebrand factor (vWF), which results in decreased levels of vWF ristocetin cofactor activity and vWF antigen.

This is reported in a variety of conditions including:

• monoclonal gammopathy of uncertain significance
• lymphoproliferative disorders
• myeloproliferative disorders

Treatment is complex and requires specialist referral.

Related topics

Australian Bleeding Disorders Registry and Haemophilia Treatment Centre Contact list [on www.ahcdo.org.au]

Back to top

Uncommon

Requires consultation with a haematologist.

Specific concentrates should be used if available.

Back to top

Absence of or reduction in the production of all or some of the cellular lines, for any reason.

May be associated with co-existing coagulation abnormalities due to the underlying condition.

Platelets may be indicated for prophylaxis or bleeding at a platelet count of:

• <10 x 109 /L in the absence of risk factors

        or

• <20 x 109 /L in the presence of risk factors (eg, fever, antibiotics, evidence of systemic haemostatic failure)

Transfusion of red cells may be appropriate depending on the patient’s clinical status and haemoglobin level.

Back to top

Patients usually present with a complex bleeding disorder associated with:

• factor deficiency (due to decreased synthesis and Vitamin K deficiency)
• thrombocytopenia (due to hypersplenism, decreased production and increased consumption)
• platelet dysfunction
• DIC
• anaemia
• primary fibrinolysis

Use of fresh frozen plasma, cryoprecipitate or platelets may be appropriate in the presence of bleeding and abnormal coagulation.

Give Vitamin K.

Desmopressin (DDAVP) may shorten the bleeding time, however the benefit is short-lived <4 hours.

Conjugated estrogens may improve the bleeding time.

Back to top

Thrombocytopenia secondary to immune-mediated platelet destruction.

Aetiology includes viral infections, drugs (eg, heparin, quinine, gold salts) associated with connective tissue disorders and idiopathic.

Usually managed by corticosteroids and/or intravenous immunoglobulin, depending on:

• the severity of the thrombocytopenia
• presence or absence of bleeding
• other patient factors.

Use of platelets is not generally considered appropriate but may be indicated in life-threatening
haemorrhage.

Related topics

Criteria for the Clinical Use of Intravenous Immunoglobulin in Australia [on www.nba.gov.au]

Back to top

Transfusion-induced thrombocytopenia secondary to immune-mediated platelet destruction, usually associated with antibodies to platelet specific antigens, most frequently anti-HPA-1a.

Give intravenous immunoglobulin.

Transfusion of antigen-negative platelets may be required.

Related topics

Criteria for the Clinical Use of Intravenous Immunoglobulin in Australia [on www.nba.gov.au]

Back to top

Platelet dysfunction results from a variety of congenital and acquired defects.

Medication is the most common cause with prostaglandin inhibitors, such as aspirin being most frequently implicated.

The bleeding tendency in platelet dysfunction defects is extremely variable and the platelet count is not a reliable indicator.

Ideally in the surgical setting antiplatelet medications should be ceased 7–10 days prior to surgery.

Use of platelets may be appropriate depending on clinical features and the clinical setting.

Back to top

Thrombocytopenia secondary to hypersplenism.

When hypersplenism exacerbates pathologic thrombocytopenia caused by another mechanism, patients sometimes become refractory to platelet transfusions even in the absence of a specific HLA or other immune-mediated platelet antibody.

Use of platelets may be appropriate depending on clinical features and the clinical setting.

Back to top

Decreased biological activity of factors II, VII, IX, X and protein C and protein S; with prolongation of the INR.

Risk factors include:

• poor diet
• malabsorption
• antibiotic use
• recent surgery
• liver or kidney dysfunction

Vitamin K given orally, subcutaneously or intravenously, depending on the clinical circumstances.

Back to top

Patients develop a mild to moderate haemostatic defect due to platelet dysfunction, abnormalities of vWF multimers and decreased platelet vWF.

The decrease in haematocrit further aggravates in vivo platelet dysfunction.

The levels of clotting factors are usually normal, and elevated levels of fibrinogen, factor VIII and vWF are typically present.

Platelet transfusion may be helpful in emergencies however the infused platelets rapidly become dysfunctional in the uraemic environment.

Red cell transfusion to achieve a haematocrit >27%.

Aggressive dialysis usually improves platelet dysfunction but incompletely corrects the haemostatic defect.

Desmopressin (DDAVP) provides effective short-term improvement of haemostasis

• Maximal at 4 hours and wears off by 6–8 hours in many patients

Repeated administration of DDAVP may lead to tachyphylaxis and hyponatremia.

Cryoprecipitate often promptly shortens the bleeding time and decreases bleeding.

High-dose conjugated estrogens may achieve a longer lasting improvement in haemostasis.

Back to top