B-cell prolymphocytic leukemia (B-PLL)

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 B-cell prolymphocytic leukemia (B-PLL) is a rare lymphoproliferative disorder characterized by the out of control growth of B-cells (B-lymphocytes), primarily involving blood, bone marrow, and spleen. Prolymphocytes are medium-sized cells with variable amount of light basophilic cytoplasm, round, oval or indented nucleus, moderately condensed chromatin, and a prominent nucleolus. B-PLL usually affects older adults and it is slightly more common in men than women. B-PLL occurs as a transformation or evolution of a more slow-growing B-cell cancer, such as chronic lymphocytic leukemia. Rarely, this is a primary disorder.

The genetic features causing B-PLL are largely unknown. B-PLL can involve deletions from chromosome 11 and chromosome 13. Many cases have been reported with breakpoints involving 14q32, particularly with t(11;140(q13;q320, which is found in up to 20%v of cases of PLL. Mutations of p53 appear in the cells of 50% of PLL patients. The prognosis of B-PLL patients is quite poor

Immunophenotype:

Strongly express surface Immnoglobulins (kappa or lambda) and surface IgM as well as B cell markers CD19, CD20, CD22, CD79a, CD79b, FMC7. CD5 can be expressed and negative for CD23.

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Reference:

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