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Primary myelofibrosis B068

Last amended 
29 June 2015
Current RMA Instruments:
Reasonable Hypothesis SOP
17 of 2013
Balance of Probabilities SOP
18 of 2013
Changes from Previous Instruments:

SOP Bulletin 164

ICD Coding:
  • ICD-9-CM Codes: 238.72, 289.8
  • ICD-10-AM Codes: D47.4

This is a blood disorder in which the bone marrow (myelo-) becomes replaced with fibrous tissue. As such the bone marrow blood cell production shifts to non-bone regions (extra-medullary haematopoiesis) of the liver and spleen leading to enlargement of these organs (hepatosplenomegaly).

The adjective ‘primary’ implies that there is no known secondary cause for this type of condition, that is it is idiopathic.  Note that whilst this is classified as a neoplasm it is not a malignant neoplasm.

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

This diagnosis is complex and is a diagnosis of exclusion of secondary causes.  Hence an opinion from a specialist haematologist is necessarily required in addition to copies of reports of a full blood count, and a peripheral blood smear and bone marrow trephine/aspirate.  Note that the condition can be asymptomatic, and picked up incidentally on an blood film or with Splenomegaly.

Are there sub-factors that require specific information? – No.
Additional diagnoses covered by SOP
  • Chronic idiopathic myelofibrosis
  • Myelofibrosis with myeloid metaplasia
Conditions excluded from SOP
  • Secondary myelofibrosis
Unconfirmed diagnosis

If, after applying the above information, you are unable to confirm the diagnosis, you should then:

  1. seek medical officer advice about further investigation, or;
  2. re-encode the condition, if appropriate.

The following investigations may be useful in establishing the diagnosis:

  • Report from treating specialist