Name

B-cell prolymphocytic leukemia

ICD-O-3 Morphology

9833/3: B-cell prolymphocytic leukemia
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

B-PLL
Prolymphocytic leukemia, B-cell type

Definition

B-cell prolymphocytic leukemia (B-PLL) is a neoplasm of B-cell prolymphocytes affecting the peripheral blood, bone marrow, and spleen. Prolymphocytes must constitute > 55% of lymphoid cells in peripheral 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 with unmutated IGH gene
IGHV3 and IGHV4

Immunophenotyping

CD5
CD19 strongly express
CD20 strongly express
CD22 strongly express
CD23
CD38 expressed
CD79a strongly express
CD79b strongly express
CD200 weakly positive
FMC7 strongly express
Strong surface IgM +/- IgD

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

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 (U.S. only)

C91.3 Prolymphocytic leukemia of B-cell type (effective October 01, 2015)

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 222-223

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
Glossary