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Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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Introducti<strong>on</strong><br />

In Irel<strong>and</strong> <strong>and</strong> Northern Irel<strong>and</strong> approximately 800 new cases <strong>of</strong><br />

malignant lymphoma, Hodgkin lymphoma (HL) <strong>and</strong> n<strong>on</strong>-Hodgkin<br />

lymphoma (NHL), are registered each year. In line with the rest<br />

<strong>of</strong> the western world, the incidence <strong>of</strong> NHLs appears to be<br />

increasing, probably by 3-5% per year <strong>and</strong>, being largely a<br />

disease <strong>of</strong> the elderly, these tumours represent an increasing<br />

cause <strong>of</strong> cancer-related death. The reas<strong>on</strong>s for increasing<br />

incidence are multiple, ranging from the increasing elderly<br />

populati<strong>on</strong>, greater accuracy <strong>of</strong> diagnosis <strong>and</strong> unknown<br />

envir<strong>on</strong>mental factors. The increase has not been noted in<br />

HL <strong>and</strong> in all subtypes <strong>of</strong> NHL <strong>and</strong> cannot solely be explained<br />

<strong>on</strong> the basis <strong>of</strong> the HIV epidemic.<br />

NHLs are a heterogeneous group <strong>of</strong> diseases which are<br />

mainly linked by their origin within the lymphoid system <strong>and</strong><br />

its different cellular comp<strong>on</strong>ents. Over 60% <strong>of</strong> NHLs are<br />

accounted for by two diseases, diffuse large B-cell lymphoma<br />

(DLBCL) which is an aggressive disease <strong>and</strong> follicular lymphoma<br />

(FL) which is an indolent or n<strong>on</strong>-aggressive entity. Many other<br />

less- comm<strong>on</strong> <strong>and</strong> <strong>of</strong>ten rare sub-types <strong>of</strong> NHL exist. All display<br />

distinct natural histories <strong>and</strong> require complex diagnostic<br />

approaches <strong>and</strong> management.<br />

The Lymphoma Forum <strong>of</strong> Irel<strong>and</strong> (LFI) first met in October 2002<br />

<strong>and</strong> outlined its aims as: the promoti<strong>on</strong> <strong>of</strong> a high st<strong>and</strong>ard <strong>of</strong><br />

care <strong>and</strong> the development <strong>of</strong> all aspects <strong>of</strong> lymphoma diagnosis<br />

<strong>and</strong> treatment in Irel<strong>and</strong> <strong>and</strong> Northern Irel<strong>and</strong>, development <strong>and</strong><br />

fostering <strong>of</strong> research initiatives relating to malignant lymphomas<br />

<strong>and</strong> interacti<strong>on</strong> with other relevant bodies towards achieving<br />

these aims. Members <strong>of</strong> the LFI include medical <strong>on</strong>cologists,<br />

haematologists, pathologists <strong>and</strong> radiati<strong>on</strong> <strong>on</strong>cologists involved<br />

in the diagnosis <strong>and</strong> management <strong>of</strong> malignant lymphomas.<br />

After a number <strong>of</strong> exploratory meetings, it was agreed that the<br />

initial step required was the development <strong>of</strong> management<br />

guidelines for these diseases in line with the WHO classificati<strong>on</strong><br />

<strong>and</strong> best internati<strong>on</strong>al treatment practices. As an initial effort,<br />

guidelines for the diagnosis <strong>and</strong> management <strong>of</strong> 22 diseases<br />

have been developed as well as guidelines for the management<br />

<strong>of</strong> some generic issues. This is the first update <strong>of</strong> the guidelines<br />

issued in parallel with the updated WHO guidelines published in<br />

2008 <strong>and</strong> these will be made available <strong>on</strong> the Haematology<br />

Associati<strong>on</strong> <strong>of</strong> Irel<strong>and</strong> website.<br />

Over the last forty years there have been numerous efforts to<br />

classify lymphoid malignancies culminating in the WHO<br />

classificati<strong>on</strong> introduced after much internati<strong>on</strong>al effort in 2001.<br />

It is clear that malignant lymphomas require a sophisticated<br />

diagnostic approach based <strong>on</strong> clinical features, morphology,<br />

immunophenotyping <strong>and</strong> genetic analysis. It is essential that<br />

such an approach underpins the clinical management <strong>of</strong> these<br />

diseases, many <strong>of</strong> which are amenable to cure. Clinical care<br />

should be delivered by those with expertise <strong>and</strong> experience<br />

in the area, within a multidisciplinary setting.<br />

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