Bleeding disorders

Introduction

Bleeding disorders may present at birth or develop later in life.
The bleeding may be spontaneous or follow trauma or surgery and may be due to defective blood vessels, platelet disorders or clotting factor deficiency.
Past episodes of excessive bleeding e.g. following circumcision, a family history of bleeding and drug therapy may be important clues to the diagnosis.
The pattern of bleeding is a helpful guide to its cause. In platelet and vessel wall defects, bleeding is usually into skin and mucosal surfaces like the gums, nose, gastrointestinal tract, whereas in coagulation factor deficiency (e.g. haemophilia), bleeding is into deep tissues like the brain, joints and muscles.
In newborns with vitamin K deficiency (which leads to multiple coagulation factor deficiency) spontaneous bleeding occurs from various sites such as the umbilical cord, gastrointestinal tract, scalp and brain.

Causes of bleeding disorders

Bleeding disorders are caused by the following:

  1. Haemophilia
  2. Von Willebrand disease
  3. Liver disease
  4. Vitamin K deficiency especially in newborns
  5. Bone marrow failure
  6. Low platelet count from any cause
  7. Disseminated Intravascular Coagulation (DIC) from any cause
  8. Drug induced:
    • Herbal preparations
    • Aspirin
    • Clopidogrel,
    • Warfarin,
    • Rivaroxaban
    • Heparin

Symptoms of bleeding disorders

The symptoms of bleeding disorders are:

  1. Spontaneous bleeding from mucous membranes or cuts
  2. Easy bruising, bleeding from orifices
  3. Excessive bleeding from cuts or incisions
  4. Deformed joints from recurrent joint bleeds
  5. Swelling at site of blood collection (pseudotumours)
  6. Pain limiting movement

signs of bleeding disorders

  1. Pallor
  2. Excessive bleeding
  3. Localised swelling due to bleeding into body spaces e.g. Joints
  4. Tenderness
  5. Limitation of movement and joint deformities
  6. Purpura, petechiae, ecchymosis

Investigations

  • FBC and blood film comment
  • Platelet count
  • Liver function tests
  • Prothrombin time, INR, partial thromboplastin time
  • Bleeding time

Treatment for bleeding disorders

Objectives

  • To prevent or arrest bleeding
  • To identify and correct underlying cause

Non-pharmacological treatment

  • Apply regulated pressure dressing and/or ice packs to minimise bleeding where possible
  • Stop any event/drugs responsible for bleeding or which may aggravate bleeding
  • Educate haemophiliacs on their disease, encouraging them to¬† minimise trauma-prone activities, and to inform doctors of their condition before any surgical procedure
  • Avoid unnecessary injections and surgical procedures in all patients (especially, those with a family history of bleeding tendencies)
  • Physiotherapy on affected joints
  • ¬†Surgery

Pharmacological treatment

A. General Measures:

Fluid replacement following acute severe blood loss
Sodium Chloride 0.9%, IV,
Adults: (See ‘Shock’)
Children and Neonates: 20 ml/kg bolus stat.
Repeat if there is no clinical improvement

And

Concentrated red cell transfusion, IV,
Adults, Children and Neonates

  • 3 ml/kg for each expected 1 g/dL haemoglobin rise

B. Bleeding due to underlying liver disease

1st Line Treatment
Evidence Rating: [A]
Vitamin K, IV,
Adults: 5-10 mg stat.
Children: 3-5 mg stat.
Neonates (irrespective of history of vitamin K injection)
Term; I mg stat
Preterm; 500 micrograms stat

And

Fresh Frozen Plasma, IV,
Adults, Children, Neonates

  • 15-20 ml/kg

C. Haemophilia A

Recombinant Factor VIII, intravenous,
1 international unit raises percentage of Factor VIII on average by 2 per kg body weight.
Required units body weight (kg) x desired factor rise x 0.5 (Repeated as needed)

Or

Desmopressin,
Intranasal, 150 microgram spray into each nostril, 12 hourly. May be repeated for 3 days

D. Haemophilia B

Recombinant Factor IX
Administration of 1 unit/kg factor IX (recombinant) generally increases factor IX levels by approximately 0.8% in adults and 0.7% in children <15 years of age

E. Von Willebrand Disease

Desmopressin,
Intranasal, 150 microgram spray into each nostril, 12 hourly. May be repeated for 3 days

F. DIC

Treat underlying cause
Fresh Frozen Plasma
Adults, Children, Neonates

  • 15-20 ml/kg

Or

Coagulation factor, red cell and platelet concentrates as needed G.

G. Thrombocytopaenia requiring platelet transfusion

Platelet Concentrate.
Adults, Children, Neonates

  • 10 ml/kg (raises platelet count by 5 x 109/L)

Prevention of local fibrinolysis 
Tranexamic Acid, oral and IV,
100 mg/kg
Then
10 mg/kg
Adults 1300 mg oral 8 hourly
2nd Line Treatment
Evidence Rating: [A]
Haemophilia A

  • Purified factor VIII
  • Cryoprecipitate, IV,

Adults, Children, Neonates

  • 1.5-2.0 packs/10 kg

Haemophilia B

  • Purified factor IX
  • Fresh frozen plasma

Referral Criteria

Refer all haemophiliacs and all patients with unexplained recurring bleeding episodes and those requiring surgery to the physician specialist or haematologist for initial assessment, treatment plan and scheduled comprehensive review.