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Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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Prognostic Factors / Index:<br />

i. Early stage disease (I – IIA)<br />

ii.<br />

Adverse factors:<br />

Advanced stage disease (IIB – IV)<br />

Adverse factors:<br />

(Internati<strong>on</strong>al<br />

Prognostic score)<br />

Age > 50 years<br />

ESR > 50 mm /hr or > 30 mm /hr<br />

with B symptoms<br />

Four or more separate nodal<br />

sites involved<br />

Mediastinial mass ratio > 1/3<br />

Age > 45 years<br />

Male sex<br />

Stage IV<br />

Haemoglobin < 10.5 g/dl<br />

Albumin < 40 g/l<br />

Lymphocytes < 0.6 x 10 9 /l or < 8%<br />

White blood count > 15 x 10 9 /l<br />

Potential Pitfalls<br />

i. Failure to differentiate between NLPHL <strong>and</strong> CHL<br />

ii. C<strong>on</strong>fusi<strong>on</strong> with DLBCL <strong>and</strong> its variants<br />

iii. Incorrect stage<br />

<strong>Treatment</strong> <strong>of</strong> nodular lymphocyte<br />

predominant HL<br />

The treatment <strong>of</strong> NLPHL is undergoing review. Where there is<br />

stage I disease <strong>and</strong> no B symptoms or adverse clinical risk<br />

factors, the patient may be treated with involved field radiati<strong>on</strong><br />

therapy (IFRT) 30Gy or may be observed following nodal excisi<strong>on</strong><br />

in the absence <strong>of</strong> residual disease. Rituximab may be used as an<br />

alternative or in additi<strong>on</strong> to radiotherapy. Patients with extensive<br />

or relapsed disease can be treated with Rituximab c<strong>on</strong>taining<br />

CHOP or ABVD.<br />

<strong>Treatment</strong> <strong>of</strong> classical HL<br />

Early stage disease<br />

Clinical stage IA or IIA<br />

<strong>Treatment</strong>:<br />

ABVD x 4 cycles <strong>and</strong> involved field radiati<strong>on</strong><br />

therapy (IFRT) 20-30Gy<br />

Or<br />

ABVD x 6 cycles<br />

The use <strong>of</strong> radiotherapy in stage I <strong>and</strong> II disease is associated<br />

with a lower risk <strong>of</strong> relapse, <strong>and</strong> this has to be <strong>of</strong>f set by<br />

c<strong>on</strong>cerns about l<strong>on</strong>g term morbidity following radiotherapy.<br />

Advanced Stage Disease<br />

Clinical stages IIB to IV<br />

St<strong>and</strong>ard treatment is with ABVD x6-8 cycles or escalated<br />

BEACOPP. A rati<strong>on</strong>al approach using the known prognostic value<br />

<strong>of</strong> PET scanning after 2 courses <strong>of</strong> ABVD may be to intensify<br />

treatment to escalated or dose-dense BEACOPP if the PET scan is<br />

deemed positive at this stage. This approach is currently being<br />

tested in the RATHL study sp<strong>on</strong>sored by the British NCRN group.<br />

Little c<strong>on</strong>sensus exists <strong>on</strong> the role <strong>of</strong> radiati<strong>on</strong> in advanced<br />

stage disease. Ideally all patients should be reviewed in an<br />

MDT setting at the beginning <strong>and</strong> end <strong>of</strong> therapy to review the<br />

indicati<strong>on</strong> for radiotherapy<br />

Resp<strong>on</strong>se Evaluati<strong>on</strong><br />

Patients should have careful clinical evaluati<strong>on</strong> with each cycle <strong>of</strong><br />

chemotherapy <strong>and</strong> treatment delays should be avoided. ABVD<br />

can be safely administered <strong>on</strong> schedule in the presence <strong>of</strong><br />

moderate neutropenia without GCSF support<br />

Resp<strong>on</strong>se evaluati<strong>on</strong> with repeat radiology <strong>of</strong> abnormal sites at<br />

presentati<strong>on</strong> is traditi<strong>on</strong>ally undertaken after four cycles <strong>of</strong> therapy,<br />

however if F18DG-PET/CT scanning is used this evaluati<strong>on</strong> is most<br />

usefully carried out after two courses <strong>of</strong> chemotherapy<br />

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