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3.7: Bleeding Disorders

Table of Contents

3.7.1 Hereditary Bleeding Disorders

Hereditary bleeding disorders includes haemophilia A and B, Von Willebrand disease

3.7.1.1 Haemophilia

Haemophilia is an inherited, X-linked lifelong bleeding disorder which affects males almost exclusively. Most frequent haemorrhage involves joints or muscles. Bleeding parttens differ with age: Infants usually bleed into soft tissues or from the mouth but as the boy grows, characterist joint bleeding becomes more common.

Haemophilia A (Factor VIII deficiency)

  • Is the most common of the hereditary clotting factor deficiencies and are caused by deficiency of factor VIII
  • The inheritance is sex linked but up to 33% of patient have no family history and result from spontaneous mutation

Clinical Features: spontaneous joint bleeding without injury, prolonged bleeding after injury, spontaneous muscle bleeding, retroperitoneal bleeding, epistaxis and easy bruising. Complication includes arthropathy and disability.

Haemophilia B (Factor IX deficiency)

  • Is due to deficiency of clotting factor IX
  • Presentation as in Haemophilia A, this is less common 20%.

Classification of Haemophilia

Haemophilia is classified as mild, moderate or severe according to the levels of circulating factor VIII or IX and indicates the expected frequency of bleeding.

Table 3.3: Classification of Hemophilia

Classification Haemophilia A Factor VIII level Haemophilia B

Factor IX level

Clinical features
Severe <1% of normal

≤ 0.01 U/ml

≤ 1% of normal

≤ 0.01U/ml

1.Spontaneous haemorrhage 2.Frequent spontaneous haemarthrosis
Moderate 2-5%of normal

0.01-0.05 U/ml

1.Haemorrhage secondary to trauma or surgery 2.Occasional spontaneous haemarthrosis
Mild 5-40%of normal 5-40% of normal 1.Haemorrhage post trauma or surgery 2. Rare spontaneous


Investigations

  • Prolonged aPTT but normal Platelets counts
  • Confirm by factor VIII or IX assay
General Management of Haemophilia

• Avoid I.M injections and use small gauge needles if necessary

• Avoid use of NSAIDs, instead use paracetamol

• Inform the patient and parents thoroughly on the problem, and provide means of alerting other medical/pharmaceutical personnel  Genetic counselling

• For Acute Bleeding episodes (RICE)

• Ice/cold pack – 5 minutes on, 10 min off – Immobilize joint with a splint

• For haemarthrosis – AVOID incising or aspiration of the affected joint. Treat by replacing the specific factor e.g factor 8 or 9 concentrate if available or FFP (10ml/kg), joint support and tabs Paracetamol for pain.


Pharmacological Treatment

Haemophilia A (Factor VIII Deficiency) no Inhibitor

Dose depends on bleeding severity Minor bleed:

D: Factor VIII 20–40IU/kg.

Major bleed:

D: Factor VIII 50–100 IU/kg

Expected response: 1IU/kg = 2% rise in factor VIII level

Half life Factor VIII: 8–24 hrs

NOTE: If there is no response to appropriate replacement therapy test for inhibitors

Haemophilia B (Factor IX deficiency) no inhibitor

Dose depends on bleeding severity

Minor bleed:

D: Factor IX 20-50IU/kg

Major bleed:

D: Factor IX 100IU/kg

Expected response: 1IU/kg= 1.5 rise in the factor IX level

Half-life Factor IX: 16–24 hrs

OR

C: Fresh frozen plasma (FFP) can be used where factor concentrate is unavailable. Average dose 10-15mls/kg

Note:

If there is no response to appropriate replacement therapy tests for inhibitors (an inhibitor is formed when one develops antibodies against factor concentrates) Detection of inhibitor is by aPTT mix study and confirmed by Bethesda assay (BU)

Factor VIII Inhibitor management Options

  • High dose factor concentrate infusion
  • Use by-pass agent like FEIBA (APCC) or NOVO seven
  • Immune tolerance induction therapy (ITI)
  • In case of emergency surgery consider plasmapheresis
  • Adjuvant antifibronolytic agents can used with either of the above
Note:
 

All patients suspected with haemophilia A or B refer to the haemophilia treatment centre or consult haematology Unit.
Children with severe haemophilia are recommended to be on low dose prophylaxis of factor concentrate

3.7.1.2 Von Willebrand Disease (VWD)

Von Willebrand Disease is inheritade disease due to deficiency of vWF and patients present with a history of easy bruising, menorrhagea, gum bleeding and spontaneous joint bleeding in severe form, this is a commonest bleeding disorder in the population especially in women.

Diagnostic Criteria

Familial history of bleeding disorder is important, aPTT and platelets normal in except in severe form.

Confirmatory test: VWF level assay.

Pharmacological Treatment

C: Tranexamic acid 500mg (PO) 8 hourly until bleeding is stopped.

OR

C: Etamsylate 500mg (PO) 8 hourly until the bleeding stop

If no response

S: Desmopresin (DDVAP) infusion 0.3µg/kg IV Max. Dose20µg.

Note:
Patient unresponsive to DDVAP may be treated with virus-inactivated vWF containing FVIII concentrate.
Never give Etamsylate or Tranexamic acid to patients bleeding per urethral

3.7.2 Acquired Bleeding Disorders/Platelet Disorders

3.7.2.1 Disseminated Intravascular Coagulation (Dic)

Disseminated intravascular coagulation (DIC) is a pathologic, excessive generation of thrombin and fibrin in the circulating blood. During the process, increased platelet aggregation and coagulation factor consumption occur this does not allow time for compensatory increase in production of coagulant and anticoagulant factors.

Diagnostic Criteria

  • Usually are related to the underlying disorder to the DIC or both
  • Bleeding manifestation,
  • Extensive organ dysfunction,
  • Shock, renal cortical ischemia, coma, delirium and focal neurological symptoms.

Non-Pharmacological Treatment

Rapid and appropriate treatment of the underlying disorder, including antibiotics for infection, surgical debridement of necrotic tissues, chemotherapy for acute leukemia, of evacuation of dead fetus; transfusion with platelets support for thrombocytopenia, fresh frozen plasma (FFP) for coagulation factor depletion and cryoprecipitate for hypofibrinogenemia.

Multifactor deficiency, Liver disease gives Fresh Frozen Plasma 10-15mls/kg until bleeding is stopped.

  • Monitor prothrombin time (PT), international normalized ratio (INR), activated partial thromboplastin (APTT), platelet count and fibrinogen.
  • Identify and treat the cause for example infection, Leukaemia especially Acute PromyelocyticLeukaemia, sereve burn, Abraptio placenta
  • Vitamin K deficiency

CAUTION: If patient is not bleeding Platelets concentrate is contraindicated. If DIC is severe enough to cause multiorgan dysfunction, management in an intensive care unit is required.

3.7.2.2 Idiopathic thrombocytopenic Purpura (ITP)

Idiopathic thrombocytopenic Purpura is an acquired disease of children and adults and defined as isolated thrombocytopenia with no clinically apparent associated condition or other causes of thrombocytopenia. The diagnosis relies on exclusion of other causes of thrombocytopenia.

Clinical feature for adult thrombocytopenia appears to be more common in young women than in young men but among older patients, the sex incidence may be equal. Most adult patient presents with a long history of Purpura, menorrhagia, epistaxis and gingival haemorrhage are more common.

Intracerebral haemorrhage occurs infrequently but is the most cause of death overt bleeding is rare unless thrombocytopenia severe (less than 10,000/µl)

Note: A palpable spleen strongly suggests that ITP is not the cause for thrombocytopenia.

Treatment

Patients who are incidentally discovered to have asymptomatic mild or moderate ITP can safely be followed with no treatment. Patients with platelet counts over 50,000/µl usually do not have spontaneous bleeding and may undergo invasive procedure. Emergenct treatment of acute bleeding caused by severe thrombocytopenia need immediate platelet transfusion is indicated in patient with haemorrhagic emergencies

Pharmacological Treatment

C: Prednisolone 1mg/kg/day (PO) for 3–6 months then taper 10mg weekly (For all patients with platelet counts below 30,000 to 50,000µl)

OR

S: IV Immunoglobulin may be given as a single dose infusion of 0.4-

1.0g/kg followed by immediately platelets transfusion

C: 1 mg/kg/day for 7 days and tapered over a week

Surgical Management

Splenectomy is indicated in patient with refractory to prednisolone.

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