Survival for these patients is generally poor, so if appropriate for the disease, directed therapy treatment should be initiated promptly.

Standard therapies include (Bewersdorf et al. 2020; Sekeres & Cutler 2014) the following:

• hypomethylating agents (HMAs azacitidine and decitabine). Azacitidine has shown a survival benefit in patients with higher risk MDS and is the mainstay of therapy, with clinical benefit and haematological responses seen even in those who don’t achieve a complete remission. Treatment is usually continued for at least 6 months and continued based on response, though dose reductions or delays may be required in some patients.
• AML induction This can be considered in those with a high blast count and who are eligible for intensive therapy.
• Haematopoietic stem cell transplantation close to the time of diagnosis, depending on the patient’s goals of Consider proceeding to transplantation soon after an optimal donor is located.
• In the interim period before transplantation, HMA therapy, AML induction chemotherapy or enrolment in a clinical trial should be considered to prevent disease progression, although the optimal pre-transplantation therapy is unknown.

Timeframe for starting treatment

For symptomatic patients with higher risk disease, a decision about disease-specific therapy should be made and treatment begun within the first six weeks of initial specialist consultation. At times, depending on disease stability and symptoms, ongoing close monitoring could be considered depending on the patient’s circumstances.

Training and experience required of the physician

Documented evidence of the physician’s training and experience (e.g. Fellow of the Royal Australian College of Physicians or equivalent) with adequate training and experience that enables institutional credentialing and agreed scope of practice in haematology.

To oversee higher risk MDS patient care, the physician should have worked or be working in a team with experience in managing MDS and AML, and specifically the use of HMAs or intensive chemotherapy.

Documented evidence of the physician training and experience, including their specific (sub-specialty) experience with MDS and procedures to be undertaken, should be available.

Cancer nurses should have accredited training in these areas:

• anti-cancer treatment administration
• specialised nursing care for patients undergoing cancer treatments, including side effects and symptom management
• the handling and disposal of cytotoxic waste (ACSQHC 2020).

Systemic therapy should be prepared by a pharmacist whose background includes adequate training in systemic therapy medication, including dosing calculations according to protocols, formulations and/or preparation.

In a setting where no haematologist or medical oncologist is locally available (e.g. regional or remote areas), some components of less complex therapies may be delivered by a general practitioner or nurse with training and experience that enables credentialing and agreed scope of practice within this area. This should be in accordance with a detailed treatment plan or agreed protocol, and with communication as agreed with the medical oncologist or as clinically required.

Health service characteristics

To provide safe and quality care for patients having systemic therapy for higher risk MDS, health services should have these features:

• critical care support
• 24-hour medical staff availability
• 24-hour high dependence or intensive care unit
• diagnostic imaging
• pathology diagnostics
• ready access to blood banking and transfusion