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Polycythaemia Vera D001

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

SOP Bulletin 164

ICD Coding:
  • ICD-9-CM Codes: 238.4
  • ICD-10-AM Codes: D45

This is a blood disorder in which the all blood cells (red cells, white cells, platelets) are excessive in number. The adjective ‘vera’ implies that this is the true disorder (primary or idiopathic) rather than a secondary disorder.

This blood disorder causes an increase in the blood viscosity which places a strain on the heart functioning and produces a tendency towards clotting (thrombosis). There is increased blood cell turnover leading to elevated nuclear waste products leading to hyperuricaemia.

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, a peripheral blood smear and bone marrow aspirate/trephine.

Are there sub-factors that require specific information? – No.
Additional diagnoses covered by SOP
  • Primary polycythaemia
  • Polycythaemia rubra vera
Conditions not covered by SOP
  • Secondary polycythaemia.
  • Relative polycythaemia.
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
Additional SOP word definitions:

Allogenic transplant – Term referring to a tissue or cell transplant from different persons from the same species. A xenograft is from a different species, such as pig.

Autoimmune – Term literally meaning ‘self’ immune. This term refers to the pathology where the body immunologically attacks itself.

Epistaxis – Term for bleeding from nose.

Evan’s syndrome – This is the name of a thrombocytopaenia and haemolytic anaemia associated with immune mechanisms and described by Evans and his colleagues in a 1951 paper in the Archives of Internal Medicine. That is this is case where the decrease in platelets is associated with a current or past decrease in red blood cells.

Graft-versus-Host disease – Disease in which the transplanted blood cells (the graft) immunologically attack the recipient (the host). Tissue transplants normally experience the threat of rejection because the body recognises the tissue as foreign, but in this case the transplanted cells are recognising the body as foreign and initiating an attack.

HLA – Acronym for Human Lymphocyte Antigen. This is the tissue marker for immunological identification (‘number plate’) in the human body, in a similar manner to the blood antigens A, B and O in blood.

Idiopathic – A disease of unknown cause. Also known as primary. Idios is Geek for own. Though there is no single identified cause, there may be recognised multiple risk factors. Note that hypertension is usually idiopathic in 90-95% of cases, but there are well recognised risk factors for this condition.

Lymphoproliferative disorder – Neoplasia of the lymph glands or lymphoid tissue of the body.

Malignancy – Term applied to a ‘bad’ cancer or neoplasm, as opposed to a ‘good’ (benign) cancer. A malignant neoplasm manifests through invasive behaviour and through production of metastases.

Melaena – Term applied to black bowel motions due to altered blood.

Mucocutaneous – Term for mucous membranes (mouth, nose, eyes, rectum, vagina) and skin.

Peripheral blood smear – This is where the blood is examined under a microscope after being smeared onto a microscope slide.

Reticuloendothelial system – This is an old classification term, used to group together mononuclear phagocyte type cells but also includes reticulum cells which form a network of fibres and the endothelial cells which line the blood vessels. The spleen is a chief element of this system. With regards to the current statement of principles, the use of reticuloendothelial system refers to the system which removes out-of-date or abnormal platelets from the blood.

Thrombocyte – Also known as a platelet, being literally a clot producing cell. However this is not really a cell being a cell fragment from the bone marrow megakaryocyte.