Diagnosis and investigation

• FBC: lymphocytosis in 36%; anaemia and mild thrombocytopenia only in very advanced disease.

• Blood film: intermediate size lymphocytes; nucleus often has clefts and indentations; smear cells unusual (cf. CLL).

• Immunophenotype: critical to diagnosis (fflp)74); SmIg strongly+ CD5+, CD10-, CD19+, CD23-, CD79b+, cyclin D1+.

• Cytogenetics: t(11;14) in 50-90% by FISH techniques.

• Bone marrow: trephine biopsy demonstrates involvement in >70% with nodular, interstitial or diffuse patterns similar to CLL.

• Lymph node biopsy: mantle zone expansion or diffuse effacement of nodal architecture by uniform 'centrocytes'; characteristic immuno-chemistry pattern: CD5+, CD10-, CD79b+, cyclin-D1+.

• Up to 30% have detectable paraprotein band, usually IgM.

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