Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-10-AM Codes: D46, C94.6

Brief description:

Myelodysplastic neoplasm (syndrome) is a group of disorders in which there is ineffective production of blood cells, dysplasia or abnormalities of blood cell precursors in the bone marrow and low blood counts (persistent cytopenias). There is an increased risk of progression to acute myeloid leukaemia (AML).

This is not a malignant neoplasm.

Confirming the diagnosis:

The diagnosis may be suspected based on the results of a Full Blood Count and peripheral blood film, but a bone marrow aspirate and biopsy is needed for confirmation.

To establish this diagnosis, consultation with a haematologist is commonly required.

Additional diagnoses covered by these SOPs

• Myelodysplastic disorder
• Myelodysplastic neoplasm with low blasts
• Myelodysplastic neoplasm with low blasts and 5q deletion
• Myelodysplastic neoplasm with low blasts and SF3B1 mutation
• Myeloperiosteal, muscular, fascial, skin, nerve or vascular damage directly caused by the displaced fractured bone
• Hypoplastic myelodysplastic neoplasm 
• Myelodysplastic neoplasm with increased blasts 
• Refactory cytopaenia with unilineage dysplasia (RCUD)
• Refractory cytopaenia with multilineage dysplasia (RCMD)
• Refractory anaemia with ring sideroblasts (RARS)
• Refractory anaemia with excess blasts (RAEB)

Conditions not covered by these SOPs

• Acute myeloblastic leukaemia*  Acute myeloid leukaemia
• Acute promyelocytic leukaemia*  Acute myeloid leukaemia
• Acute myelomonocytic leukaemia*  Acute myeloid leukaemia
• Acute panmyelosis with myelofibrosis*  Acute myeloid leukaemia
• Aplastic anaemia*
• Chronic neutrophilic leukaemia #
• Chronic eosinophilic leukaemia #
• Essential thrombocytopenia *
• Immune thrombocytopaenic purpura*        
• Juvenile myelomonocytic leukaemia 
• Myelodysplastic/myeloproliferative neoplasm wit neutrophilia #
• Myelodysplastic/myeloproliferative neoplasm with SF3B1 mutation #
• Myelodysplasia of the spinal cord #
• Myelodysplastic/myeloproliferative neoplasm not otherwise specified #
• Myeloid sarcoma*  Acute myeloid leukaemia
• Polycythaemia vera *
• Primary myelofibrosis *
• Thrombocytosis * Essential thrombocythaemia                              

* another SOP applies  - the SOP has the same name unless otherwise specified

^# non-SOP condition

Clinical onset

The clinical onset of myelodysplastic neoplasm (MDS) typically occurs at or shortly before the time of diagnosis and is often asymptomatic, being discovered incidentally through routine blood test conducted for unrelated reasons. When symptomatic, it may present with features related to deficiencies in blood cell lines- anaemia (fatigue, weakness, shortness of breath or pallor); and neutropenia, resulting in recurrent or atypical infections. The severity of symptoms depends on the extent of cytopaenias and disease progression, and systemic symptoms such as fever or weight loss may appear in advanced stages. 

Clinical worsening

The natural history of myelodysplastic syndrome is to worsen or to eventually transform to acute myeloid leukaemia (AML).  Such a transformation represents new onset of AML rather than worsening of MDS. There are no clinical worsening factors in the SoP apart from inability to obtain appropriate clinical management.  Early detection and treatment can be effective in controlling symptoms and prolonging survival, so lack of appropriate treatment could result in a worsening of the condition.

Comments on SOP factors

Undergoing treatment with radioactive iodine or phosphorus does not include diagnostic tests where these agents are used as tracers.