Guidelines on Diagnosis and Treatment of Malignant Lymphomas
Guidelines on Diagnosis and Treatment of Malignant Lymphomas
Guidelines on Diagnosis and Treatment of Malignant Lymphomas
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Mature B-Cell Neoplasms<br />
Chr<strong>on</strong>ic Lymphocytic Leukaemia/<br />
Small Lymphocytic Lymphoma<br />
Definiti<strong>on</strong> <strong>and</strong> Incidence:<br />
Chr<strong>on</strong>ic lymphocytic leukaemia / small lymphocytic lymphoma<br />
(CLL/SLL) is a neoplasm <strong>of</strong> m<strong>on</strong>omorphic small, round<br />
B- lymphocytes in the peripheral blood, b<strong>on</strong>e marrow <strong>and</strong> lymph<br />
nodes admixed with prolymphocytes <strong>and</strong> para-immunoblasts<br />
expressing CD5 <strong>and</strong> CD23. The term SLL is restricted to cases<br />
with the tissue morphology <strong>and</strong> immunophenotype <strong>of</strong> CLL but<br />
without a leukaemic comp<strong>on</strong>ent. CLL comprises 90% <strong>of</strong> chr<strong>on</strong>ic<br />
leukaemias in the USA <strong>and</strong> Europe <strong>and</strong> 7% <strong>of</strong> NHLs present as<br />
CLL/SLL. The majority <strong>of</strong> patients are >50 years old (median<br />
age 65) <strong>and</strong> the M: F ratio is 2:1. The incidence is 0.72cases<br />
/100,000 per year.<br />
ICD – O Codes: CLL 9823/3<br />
B-SLL 9670/3<br />
Clinical presentati<strong>on</strong><br />
Most patients with CLL are asymptomatic <strong>and</strong> the disease is<br />
diagnosed incidentally <strong>on</strong> routine full blood count. Presenting<br />
features can include lymphadenopathy, fatigue, auto-immune<br />
haemolytic anaemia, infecti<strong>on</strong>, or evidence <strong>of</strong> b<strong>on</strong>e marrow failure.<br />
Pathology<br />
The lymphoid infiltrate effaces normal lymphoid architecture<br />
with a pseudo-follicular pattern <strong>of</strong> regularly distributed<br />
pale areas c<strong>on</strong>taining larger cells in a dark background <strong>of</strong> small<br />
cells The cells are slightly larger than normal lymphocytes, have<br />
a high nuclear-cytoplasmic ratio, round nucleus <strong>and</strong> occasi<strong>on</strong>al<br />
small nucleolus. The pseud<strong>of</strong>ollicles c<strong>on</strong>tain a c<strong>on</strong>tinuum <strong>of</strong><br />
small, medium <strong>and</strong> large cells i.e. lymphocytes, prolymphocytes<br />
<strong>and</strong> para-immunoblasts. The size <strong>of</strong> the pseud<strong>of</strong>ollicles <strong>and</strong><br />
the number <strong>of</strong> para-immunoblasts vary but there is no<br />
well-documented correlati<strong>on</strong> between histological findings<br />
<strong>and</strong> clinical outcome.<br />
Cell morphology can vary <strong>and</strong> may be c<strong>on</strong>fused with mantle cell<br />
lymphoma (MCL). Plamacytoid differentiati<strong>on</strong> may also be present.<br />
In the blood <strong>and</strong> b<strong>on</strong>e marrow similar small lymphocytes are<br />
found <strong>and</strong> smudge, smear or basket cells are typically seen <strong>on</strong><br />
blood films. Prolymphocytes, which are larger cells with a<br />
prominent nucleolus, usually account for
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