ITP and B-cell Lymphoma Feature in Latest BSH Guidance

Liam Davenport

April 05, 2019

GLASGOW — Recommendations on how to prevent glucocorticoid-induced osteoporosis in immune thrombocytopenia (ITP) patients receiving steroids and the optimal management of primary mediastinal B-cell lymphoma are just two of the numerous guidance documents published by the British Society for Haematology (BSH) in the past year.

Since the 2018 BSH Annual Scientific Meeting in Liverpool, the Society has published 12 sets of guidelines and good practice papers in general haematolgy, haemato-oncology, haemostasis and thrombosis, and transfusion.

This includes two Good Practice Papers since the start of 2019, designed to recommend best practices in areas where there is limited evidence but patient care will benefit from expert consensus.

Good Practice

The first looks at the prevention of glucocorticoid-induced osteoporosis in patients with ITP receiving steroids, and makes recommendations on who are the most at-risk patients, as well as how to approach prevention with lifestyle and/or dietary interventions.

The second, which addresses the management of primary mediastinal B-cell lymphoma, emphasises the importance of offering clinical trials to patients, in addition setting out the standard of care chemo-radiotherapy regimens.

The Society also publishes full guidelines, which are evidence based and developed following a professional literature review, and position papers for the adoption and adaptation of non-UK evidence-based guidelines.

Dr Shubha Allard, is a consultant haematologist for Barts Health NHS Trust, London, and NHS Blood and Transplant, and chair of the BSH Guidelines Committee.

Speaking after a session devoted to BSH guidance at this year's  BSH Annual Scientific Meeting in Glasgow, she told Medscape News UK that the Committee has four task forces that focus on different areas within the field, comprising clinicians, scientists and, where relevant, nurses, all of whom are working in day-to-day practice.

Jo Howard, clinical lead for Haematology at Guy's and St Thomas' NHS Foundation Trust, London, and vice chair of the Guidelines Committee, added that the task forces meet quarterly, at which point they discuss any "holes" in terms of recommended practice.

One of the aims of developing the various guidelines, good practice and position papers is, at least in part, to tackle variations in practice.

Dr Allard said: "Where you’ve got a good evidence base it’s easier to come up with a very strong recommendation supported by randomised controlled trials, but where you haven’t got the evidence it’s much harder."

She added: "But the whole purpose of the guidelines is trying to reduce that variation, and linked to the guidelines we would always recommend an audit as a quality improvement exercise."

Dr Howard pointed out that, even in the absence of a strong evidence base, having a good practice paper, while based primarily on expert opinion, "is also really valuable for the practising haematologist".

For Dr Allard, this is partially about raising awareness, "because lack of knowledge about the guidelines should never be an excuse".

"It’s then how you utilise that within your own hospital, and we want to produce some educational tools so that people have got the means to disseminate the guideline."

Glucocorticoid-Induced Osteoporosis

Dr Quentin Hill, from Leeds Teaching Hospitals, Leeds, and colleagues, developed the good practice paper on glucocorticoid-induced osteoporosis following a search of the Ovid Medline, Embase and Cochrane databases.

Along with colleagues from the UK ITP Forum and the International Osteoporosis Foundation, a series of recommendations for the prevention of osteoporosis in all ITP patients receiving glucocorticoids was set out.

They state that all men and women aged ≥70 years, and men aged ≥50 years and postmenopausal women with a previous fragility fracture should be considered for prevention, while other age groups should be assessed for FRAX score at the start of treatment.

Among other recommendations, the paper says that all adults starting glucocorticoids should be given lifestyle advice, and have their serum calcium and vitamin D levels checked.

Adequate daily vitamin D intake is defined as 800 iu while for calcium it is 700–1200 mg, which can be achieved through diet, if possible, or supplements, if needed.

In patients at high risk of fracture, oral alendronate or risedronate should be considered. If poorly tolerated or contraindicated, zoledronic acid, denosumab or teriparatide are appropriate alternatives.

Primary Mediastinal B-cell Lymphoma

In their good practice paper on primary mediastinal B-cell lymphoma management, Dr Kate Cwynarski, University College London Hospitals NHS Foundation Trust, looked at the presentation and staging of the disease, as well as issues in fertility and in pregnancy.

When treating the disease, they recommend that patients be offered participation in a clinical trial "wherever possible", while stating that six cycles of R-CHOP chemotherapy plus involved site radiotherapy (ISRT) is the standard of care.

While six cycles of the DA-EPOCH-R regimen without ISRT can be offered as an alternative, the authors underline that it "should only be given in centres experienced in the delivery of complex chemotherapy regimens".

Patients with relapse or refractory disease may be considered for radiotherapy if it was not used initially and the relapse is localised.

However, the authors restate the recommendation that, where possible, patients be offered a clinical trial.

For salvage therapy, the regimen should be the same as that used in the treatment of relapsed diffuse large B-cell lymphoma.

Genetic Haemochromatosis

For Dr Allard, however, one of the most clinically relevant guidelines published by the BSH since their last Annual Scientific Meeting in Liverpool came out almost a year ago.

In April 2018, Dr Edward Fitzsimons, honorary associate clinical professor at Glasgow University, and colleagues published an update to the BSH guideline on the diagnosis and therapy of genetic haemochromatosis.

Presenting the revised guidelines at this year’s meeting, Dr Fitzsimons said that homozygosity for the condition is so prevalent in the UK that it must be "the most common condition that people have never heard of".

He said that the need for updated guidelines on genetic haemochromatosis is underlined by there being variation in practice when it comes to treatment not just regionally but also within cities and even within hospitals.

The guidlines emphasise the importance assessing for potential complications, such as liver fibrosis and cirrhosis, bronzed diabetes and other endocrine disorders, arthropathy and cardiac toxicity.

Venesection remains the mainstay of treatment, with the frequency individualised to the patient, "taking into account the presenting severity of iron overload, organ damage, age, comorbidities, and tolerability of the procedure".

For some patients, the guidelines state, the need for venesection will decrease over time, while others may have more rapid iron re-accumulation, particularly if they had marked iron loading at presentation.

No funding declared.

The BSH paid the expenses incurred during the writing of the guidance. Hill is secretary of the UK ITP Forum and has received honoraria from Novartis and Shire.

BSH 2019: Guidelines. Presented April 2.

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