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SOP Bulletin 236

ICD Coding

ICD-10-AM Codes: D66, D67, D68.1

Brief description

This is a genetic disorder of coagulation factors VIII (haemophilia A), IX (haemophilia B), or XI (haemophilia C), causing abnormal clotting of blood.  It is an X chromosome-linked disorder and so mostly affects males.

Confirming the diagnosis

This diagnosis is made based on blood tests such as clotting time, prothrombin time, activated partial thromboplastin time (APTT), factor 8 assay and factor 9 assay.  Genetic testing is performed in most patients.

The relevant medical specialist is a haematologist.

Additional diagnoses covered by SOP

• Haemophilia A (factor VIII) aka classical haemophilia
• Haemophilia B (factor IX) aka Christmas disease
• Haemophilia C (factor XI) aka Rosenthal’s disease

Conditions not covered by SOP

• Von Willebrand disease*
• Acquired haemophilia^#

* Another SOP applies

^# Non-SOP conditions 

Clinical onset

The condition is typically diagnosed very early in life (by age 3) and presents with signs of abnormal clotting (e.g. easy bruising).

Clinical worsening

The only SOP factor is for inability to obtain appropriate clinical management.

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 factor VIII or factor IX and can be classified as severe, moderate, or mild.  Patients with mild disease experience infrequent bleeding that is usually secondary to trauma.  Among those with residual factor VIII or factor IX 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 are unlikely to be compatible with military service.