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Introduction

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Prolymphocytic leukaemia (PLL) is a rare lymphoproliferative disorder resulting from a clonal proliferation of mature B lymphocytes with specific cytological features [1]. Prognosis is generally poor but a more indolent form of the disease has also been recognized [2].

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Clinical features

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The disease affects mainly the elderly and is more common in men than in women. Splenomegaly is characteristic with lymphadenopathy usually being minor.

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Haematological and pathological features

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The white cell count is often high and about half of patients have anaemia and thrombocytopenia. In one study prolymphocytes constituted more than 55% of circulating cells and this was suggested as a diagnostic cut-off point [3]. However, it should be noted that a large proportion of prolymphocytes is also occasionally observed in advanced chronic lymphocytic leukaemia (CLL) and this cut-off point is therefore arbitrary. Prolymphocytes are medium to large lymphoid cells with a single prominent nucleolus (Figures 4.1, 4.2, 4.3); perinucleolar chromatin condensation gives the nucleolus a vesicular appearance. In some patients cells are fairly uniform in size whereas in others there is a range from medium to large. When smaller cells are present, their nucleoli are usually smaller and less prominent.

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Figure 4.1

Peripheral blood film from a patient with PLL showing one smear cell and a fairly uniform population of medium sized lymphoid cells with large prominent nucleoli. Romanowsky stain, x 60 objective.

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Figure 4.2

Peripheral blood film from a patient with PLL showing mainly medium sized prolymphocytes and one very large cell. Romanowsky stain, x 100 objective.

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Figure 4.3

Peripheral blood film from a patient with PLL showing two prolymphocytes, one of which is binucleated. Romanowsky stain, x 100 objective.

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The trephine biopsy shows an interstitial/nodular or diffuse pattern of infiltration. Lymph node infiltration is diffuse, sometimes with a vaguely nodular pattern. Splenic infiltration is in both red and white pulp and in the white pulp it may be nodular (Figures 4.4 and 4.5).

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Figure 4.4

Section of spleen from a patient with PLL showing prominent white pulp infiltration. H&E, x 10 objective.

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Figure 4.5

Section of spleen from a patient with PLL showing large cells with large nuclei containing prominent eosinophilic nucleoli. H&E, x 100 objective.

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A low concentration paraprotein, most often IgM, may be present.

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Immunophenotype

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The immunophenotype (Figure 4.6) is often ...

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