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Haemophilia P010

Document
Last amended 
30 June 2015
Current RMA Instruments:
Reasonable Hypothesis SOP23 of 2015
Balance of Probabilities SOP 24 of 2015
Changes from Previous Instruments:

SOP Bulletin 179

ICD Coding:
  • ICD-9-CM Codes: 286.0, 286.1, 286.2
  • ICD-10-AM Codes: D66, D67, D68.1

This is a genetic disorder affecting coagulation factors 8, 9 or 10 causing abnormal clotting of blood.

Is specific diagnostic evidence required to apply the SOP? – Yes.

This diagnosis requires an opinion from a specialist haematologist together with specific diagnostic blood tests being a clotting time, prothrombin time, activated partial Thromboplastin time (APTT), factor 8 assay, factor 9 assay.

Are there sub-factors that require specific information? – No.

The only factor is for clinical worsening from inability to obtain appropriate clinical management.  It is difficult to ascertain that there is clinical worsening in an intermittent disorder such as this.  See notes below.

Additional diagnoses covered by SOP
  • Haemophilia A (factor 8) aka classical haemophilia (ICD-9 286.0; ICD-10 D66)
  • Haemophilia B (factor 9) aka Christmas disease (ICD-9 286.1; ICD-10 D67)
  • Haemophilia C (factor 11) aka Rosenthal’s disease (ICD-9 286.2; ICD-10 D68.1)
Conditions not covered by SOP
  • Von Willebrand’s disease
  • Acquired haemophilia
Unconfirmed diagnosis

If, after applying the above information, you are unable to confirm the diagnosis, you should then seek medical officer advice about further investigation.

Notes on clinical worsening

Worsening beyond the normal progression of the disease needs to be evident before a clinical worsening factor can be considered.

It is the nature of this disease to suffer from episodes of uncontrolled bleeding due to inadequate clotting. As such, suffering an episode due to a military related activity is not an aggravation. The aggravation / permanent clinical worsening of any intermittent condition, requires that the frequency of the episodes be increased, the severity of the episodes be increased, or death occurs.

Haemophilia is treated after diagnosis by replacing the defective blood clotting factors and avoiding the use of platelet aggregation drugs such as aspirin.

The disease manifests differently depending on the individual genetic expression. Clinically, haemophilia A and haemophilia B are indistinguishable. The disease phenotype correlates with the residual activity of FVIII [factor 8] or FIX [factor 9] and can be classified as severe (<1%), moderate (1-5%), or mild (6-30%).  Patients with mild disease experience infrequent bleeding that is usually secondary to trauma. Among those with residual FVIII or FIX activity >25% of normal, the disease is discovered only by bleeding after major trauma or during routine presurgery laboratory tests.   In the severe and moderate forms, the disease is characterised by bleeding episodes into the joints (haemarthroses), soft tissues, and muscles after minor trauma or even spontaneously.

Moderate and severe haemophilia is unlikely to be compatible with military service.