Bleeding disorders

Clinical Conditions

Haemostatic defect

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.

Ways to correct the haemostatic defect

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

Replacement therapy with recombinant factor VIII (Advate 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]

 

Haemostatic defect

Development of factor VIII autoantibodies resulting in decreased levels of coagulation factor VIII.

Associated with the post-partum state, connective tissue disorders and malignancies.

About 50% are idiopathic.

Ways to correct the haemostatic defect

Treatment is complex and requires specialist referral.

Related topics

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

Haemostatic defect

Absence/low levels of coagulation factor IX.

Ways to correct the haemostatic defect

Replacement therapy with recombinant factor IX (Benefix, Rixubis) 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]

Haemostatic defect

The most common of the inherited bleeding disorders due to a quantitative and/or qualitative defect in von Willebrand factor (vWF) protein.

The diagnosis and evaluation of treatment options are not straightforward, and requires measurement of the plasma levels of both factor VIII and vWF protein.

Ways to correct the haemostatic defect

Desmopressin (DDAVP)

Replacement therapy with plasma-derived factor VIII concentrates (Biostate) containing high molecular weight vWF multimers.

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

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

Haemostatic defect

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

Ways to correct the haemostatic defect

Treatment is complex and requires specialist referral.

Related topics

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

Haemostatic defect

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.

Ways to correct the haemostatic defect

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.

Haemostatic defect

The cause is multifactorial, which includes:

  • decreased coagulation factor levels (due to activation from contact with the extracorporeal circuit and oxygenator)
  • thrombocytopenia (probably related to the length of the procedure and hypothermia)
  • platelet dysfunction (due to the use of heparin during the procedure and other anti-platelet drugs)
  • fibrinolytic activation and haemodilution

Repeat operations are a major risk factor for postoperative bleeding.

Ways to correct the haemostatic defect

Use of platelets or fresh frozen plasma may be appropriate in the presence of bleeding and abnormal coagulation. In this situation, the platelet count is not a reliable indicator.

Haemostatic defect

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

Ways to correct the haemostatic defect

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.

Haemostatic defect

Thrombocytopenia secondary to immune-mediated platelet destruction.

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

Ways to correct the haemostatic defect

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]

Haemostatic defect

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

Ways to correct the haemostatic defect

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]

Haemostatic defect

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.

Ways to correct the haemostatic defect

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.

Haemostatic defect

The absence of a significant and sustained rise in the platelet count following platelet transfusion due to a variety of immune and non-immune factors

Ways to correct the haemostatic defect

Management is dependent on the clinical status of the patient and the aetiology of the refractoriness.

Haemostatic defect

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.

Ways to correct the haemostatic defect

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

Haemostatic defect

Cause systemic fibrinolysis.

Haemorrhage complicating these agents is most commonly localised (eg, at the site of catheterisation in the groin).

Ways to correct the haemostatic defect

Control localised bleeding with pressure packs.

Cryoprecipitate or fresh frozen plasma can be given for life-threatening bleeding.

Haemostatic defect

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

Risk factors include:

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

Ways to correct the haemostatic defect

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

Haemostatic defect

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.

Ways to correct the haemostatic defect

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.