B-cell prolymphocytic leukemia

Name
B-cell prolymphocytic leukemia
ICD-O-3 Morphology
9833/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

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Grade
6 - B-cell
Module Rule
None
Alternate Names
B-PLL
Prolymphocytic leukemia, B-cell type
Definition
More aggressive, but still treatable form of chronic lymphocytic leukemia. The malignant B cells are larger than average. The name is commonly abbreviated B-PLL. It can involve deletions from chromosome 11 and chromosome 13. It has a relatively poor prognosis.

Malignancy of B-prolymphocytes medium-sized, round lymphoid cells with prominent nucleoli, moderately condensed nuclear chromatin, relatively small amount of faintly basophilic cytoplasm, affecting blood, bone marrow and spleen. Prolymphocytes must exceed 55% of lymphoid cells in blood.
Abstractor Notes
This neoplasm affects the peripheral blood (PB), bone marrow (BM) and spleen. Prolymphocytes must exceed 55% of lymphoid cells in PB. The leukemic cells are found in the PB, BM and spleen.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear
Genetics Data
Ig genes clonally rearranged, t(11;14)(q13;q32) in 20%, abnormalities of p53 in 53%, del at 11q23, del at 13q14
Immunophenotyping
CD5
CD19
CD20
CD22
CD23
CD79a
CD79b
FMC7
Strong surface IgM +/- IgD
Treatments
Chemotherapy
Hormone
Radiation
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.3 Prolymphocytic Leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.3 Prolymphocytic leukemia of B-cell type
Signs and Symptoms
Progression and Transformation
None
Epidemiology and Mortality
Age: 65-69 years median age
Sex: no male or female predominance
Survival: 30-50 months median survival