B-cell prolymphocytic leukemia (B-PLL)

Epidemiology and Etiology:

Common sites:

Gross features:

Histologic features:

Immunophenotype:

Marker:

Sensitivity:

Specificity:

Molecular features:

• Not associated with survival so far
• Immunoglobulin genes clonallry rearranged (50%)
• Unmutated heavy chain gene
• All have been reported to use VH3 (68%) and VH4 (32%) gene families
• Complex karyotype commonly
• TP53 deletions (50%)
• Associated with TP53 mutations
• Deletions at 13q14 (27%)
• Uncommonly trisomy 12
• No t(11;14)(q13;q32) by definition
• These cases are considered leukemic MCL

Other features:

• Poor prognosis (median survival 30-50 months)
• No genetic changes associated with survival so far

References:

• WHO Blue Book (2008)