Reasonable Hypothesis SOP [1] | 89 of 2021 |
Balance of Probabilities SOP [2] | 90 of 2021 |
SOP Bulletin 224 [3]
Primary myelofibrosis is one of the chronic myeloproliferative disorders, in which there is chronic proliferation of types of myeloid cells in the bone marrow. A feature of this condition is the development of bone marrow fibrosis (due to proliferation of fibroblasts). This leads to impaired production of red blood cells and severe anaemia. Blood cell production then shifts to the spleen and liver, leading to enlargement of those organs (splenomegaly / hepatomegaly).
This condition is now classified as a malignant neoplasm.
This diagnosis is complex and is based on clinical and laboratory findings. Bone marrow biopsy is generally necessary to demonstrate marrow fibrosis and exclude other causes such as malignant bone marrow infiltration. Genetic testing for gene mutations may also be performed.
The relevant medical specialist is a haematologist.
* another SOP applies
# non-SOP condition
The usual presentation is with severe fatigue (due to anaemia). The condition may also first present with symptoms or signs of an enlarged spleen. It may also be found following detection of an enlarged liver or abnormal blood test findings.
The only SOP factor is for inability to obtain appropriate clinical management. Treatment varies with the presentation and stage of disease, but disease modifying treatment is available and appropriate in some cases, particularly in the form of transplantation.
Links
[1] http://www.rma.gov.au/assets/SOP/2021/767d06e1fc/089.pdf
[2] http://www.rma.gov.au/assets/SOP/2021/44ff530dfd/090.pdf
[3] https://clik.dva.gov.au/system/files/media/SOP%20Bulletin%20224.pdf